A system of practical medicine. By American authors. Vol. 3 (part 5 of 8)

A system of practical medicine. By American authors. Vol. 3 (part 5 of 8)

4. Brown atrophy is a very common degenerative change in the heart-muscle, particularly in the hypertrophied organ of valve affections. In old people and in persons dead of wasting diseases it seems invariably present. When advanced, the color of the muscle is quite distinctive--a dark red-brown and the consistence may be greater than normal. Microscopically, the fibres present a central accumulation of brown pigment, generally arranged about the nuclei and extending up and down the cells. The cement-substance between the cells is often unusually distinct in these cases, and seems more fragile than in healthy muscle. The composition of the pigment has not, so far as I know, been determined, but it is doubtless, like that of the brown induration of the lung and red atrophy of the liver, derived from the hæmoglobin, and possibly, as in these latter conditions, is connected with feeble venous circulation.

5. Amyloid degeneration of the heart is occasionally met with, but rarely in so advanced a grade as to be recognizable macroscopically. It occurs in the intermuscular connective tissue and in the blood-vessels, not in the fibres, and occasionally may be extensive, as in a case mentioned by Ziegler.[30]

[Footnote 30: Pathologische anatomie, 3te Aufl., Lief. i., §59.]

6. The hyaline degeneration of Zenker is sometimes seen in the heart-muscle in cases of prolonged fever. The affected fibres are swollen, homogeneous, translucent, and the striæ very faint or entirely absent.

7. Calcareous degeneration may occur in the myocardium, involving the fibres and forming a definite calcareous infiltration of the protoplasm, as well figured and described by Coats.[31] It is a rare condition, whereas extensive calcified plates in endo- and pericardium are by no means uncommon.

[Footnote 31: Pathology, 1883.]

{617} Spontaneous Rupture of the Heart.

Laceration of the wall of the heart is usually associated with fatty infiltration or degeneration, most frequently the latter. It is doubtful if in any instance the healthy muscle has broken. Rare causes are--acute softening, in consequence of embolism of a branch of a coronary artery; abscess from pyæmia; or an acute ulcer of the endocardium. Cysts simple or hydatid are mentioned, but the extreme rarity of causes other than fatty changes may be inferred from the statistics of Quain,[32] who states that of 100 cases of rupture collected by him, fatty degeneration was noted (microscopically) in 77, and in the others there was softening in all but 2, or no mention was made of the condition of the wall.

[Footnote 32: Loc. cit.]

Males are more frequently the subject of this accident than females, and the great majority of cases occur in persons over sixty years of age--two-thirds of the eases tabulated by Quain.[33]

[Footnote 33: Loc. cit.]

The rent may occur in any of the chambers, but the most frequent site is the left ventricle on the anterior wall, not far from the septum. Statistics give, for 55 cases,[34] 43 in left ventricle, 7 in right ventricle, and 3 in right auricle and 2 in the left auricle.

[Footnote 34: Elleaumé, Essai sur les Ruptures du Coeur, Paris, 1857.]

The break is generally a ragged, irregular rent in the course of the fibres, and the trajét may be oblique and crossed by strands of muscle. The internal orifice may be larger than the external; the opposite is rarely the case. Two or more rents have been found. Usually the fissure is not very long--from a quarter of an inch to an inch--but there are cases of long rents extending from base to apex. Clots usually block the orifices, and the pericardium also contains large coagula. Evidence is sometimes found to indicate that the tear has occurred slowly, as attempts at repair may be present.

The wall in the vicinity of the break has usually been found in a state of degeneration, and we can readily understand how sudden and violent contractions might strain a weak part and tear the substance. Perhaps irregularity in the contractions may be an important factor, such as we may suppose occurs when a wave of contraction reaches a patch of advanced fatty change or softening from embolism.

The accident usually takes place during exertion or excitement. Many cases are reported during straining at stool, others while lifting weights, running, or during coitus. Cases are mentioned as occurring during sleep or while at rest.

There may be no preliminary symptoms, and without warning the patient falls, and with a few gasps or a cry is dead. This occurred in 71 of the 100 cases collected by Quain. In other instances there is great pain in the præcordial region, a sense of suffocation and anguish, with vomiting, and life may be prolonged several hours. In one instance the patient lived eleven days.[35] Probably in such cases there is a small rent at first which gets blocked with clots, and only a small amount of blood oozes into the pericardium with each systole. The symptoms may be those of simple heart failure, as in a case I examined for Burland of Montreal, in which the patient lived thirteen hours after the onset of the symptoms, and was able, though with difficulty, to continue his walk up a rather steep hill.[36] Death appears to occur from shock or syncope, sometimes from compression of the heart by the extravasated blood. In the case just mentioned the amount of blood in the pericardium was {618} very much less than I have seen in cases of rupture of an aneurism into this sac.

[Footnote 35: Barth, Archiv. générales, 1871.]

[Footnote 36: This was a case which illustrated well the latency of many cases of fatty heart. The patient was an active merchant, aged sixty, who had never complained of cardiac trouble, and had only a short time before his death effected a reinsurance upon his life for a large amount.]

In protracted cases the nausea and vomiting may for a short time lead to the supposition that the case is one of severe indigestion, but, as mentioned above, in the great majority of cases death occurs at once, and in the others there can rarely be any question of diagnosis, and still less of treatment.

Atrophy of the Heart.

DEFINITION.--A diminution in size and weight of the organ, due to degeneration and atrophy of the muscular fibres.

The old writers applied the term phthisis of the heart to this condition. The decrease is always in weight, and usually in size; it is doubtful if there is an atrophic and dilated heart in which, with the wasting, the size is maintained by the dilatation. In many of the degenerations, particularly fatty and fibroid, there is local atrophy of the muscle-fibres and yet the weight and size of the organ are not changed.

The varieties which have been recognized correspond to those of hypertrophy--viz. the simple, eccentric, and concentric forms, but the two latter are probably only conditions of contraction or dilatation in a wasted heart. The post-mortem contraction in the small left ventricle of persons dead of chronic disease may be excessive; and here, as in concentric hypertrophy, the examination must be made with care.

ETIOLOGY.--The atrophy is either congenital or acquired. The congenital atrophy which is most frequently seen in women is in association with defective development of the arterial system and the generative organs. This is occasionally very marked in chlorosis, and is described and figured by Virchow in his monograph on this subject.[37] But apart from this general hypoplasia of the heart and vessels in women, we sometimes in the post-mortem room find in a man, dead perhaps of an acute disease and without any cardiac symptoms, a heart small out of all proportion to the size and general nourishment of the body. Many of the older writers mention this. Gowers refers to a case which Allan Burns narrates, in which the heart of an adult was not larger than that of a child of six or seven. Morgagni has a similar observation.

[Footnote 37: Ueber die Chlorose, Berlin, 1872.]

The great majority of the cases are secondary or acquired, and are met with in the wasting diseases, as cancer, phthisis, prolonged suppuration, and diabetes. The cardiac wasting is part of the general marasmus which affects the whole body. In about half the cases of phthisis the heart is small.[38] In cancer of the pylorus the most extreme wasting has been found. Disease of the coronary arteries is an occasional cause, but it most frequently produces local atrophy or degeneration. Compression by pericardial effusion, fatty infiltration, and pericardial adhesions are mentioned as rare causes.

[Footnote 38: Quain, loc. cit.]

A rough guess at the proportional size of the heart may be made by comparing it with the closed right fist of the person. Weighing gives the most accurate test, and in each instance regard must be had to the size of the body. In some instances the organ has weighed only two or three ounces. The heart figured by Bramwell,[39] one of the smallest on record, weighed only 2 ounces and 2 drachms. Quain[40] refers to one, from a girl aged fourteen, which weighed only 1 ounce 14 drachms.

[Footnote 39: Diseases of the Heart, 1884.]

[Footnote 40: Loc. cit.]

Usually, in secondary atrophy, the visceral pericardium is wrinkled and the coronary arteries prominent and tortuous--two features of great importance in determining atrophy and in distinguishing between the acquired and {619} congenital forms. The pericardial fat is variable in amount. Microscopically, brown atrophy is the most constant change; fatty degeneration much less common. Senile atrophy may present very similar appearances. The heart may be tough and firm from an increase in the fibrous elements. The pericardial fluid I have often noticed to be much increased.

There are no characteristic SYMPTOMS. The heart-muscle may be able to fulfil the requirements of the wasted frame. A feeble impulse and diminished area of dulness may be present, but in the marasmus of middle-aged or elderly people emphysema of the anterior margin of the lung may seriously interfere with a proper examination. The increased pericardial effusion occurs toward the end. The heart sounds are feeble and the pulse weak. Palpitation is frequent, and there may be the usual signs of anæmia, dizziness, etc.

The condition may be suspected, but is rarely diagnosed during life.

The PROGNOSIS depends upon the disease to which the atrophy is secondary, to the amelioration of which also the treatment must be directed.

Hypertrophy of the Heart.

DEFINITION.--An increase in the size of the heart due to an increased thickness, total or partial, of the muscular walls.

VARIETIES.--Two forms may be recognized--simple hypertrophy, in which the cavity or cavities remain of the normal size; and eccentric hypertrophy, in which with increased thickness of the walls there is enlargement of the cavities. Dilated hypertrophy and hypertrophy with dilatation are terms by which the latter form is most frequently described.

By many writers a third variety, concentric hypertrophy, is recognized, in which there is diminution in the size of the cavity with thickening of the walls; but in these cases we have to deal with a post-mortem change--rigor mortis; and if the organ is kept for twenty-four hours or soaked in water, the so-called concentric hypertrophy will usually disappear.

The increased size may affect the entire organ, general hypertrophy; or only one side or one cavity, partial hypertrophy. The latter is the most common. Of the single chambers the left ventricle is most frequently involved, then the right. The auricles are rarely affected alone, but the left is more often than the right.

ETIOLOGY.--Disturbed innervation and increased work are the two principal causes of cardiac hypertrophy. We see hypertrophy from deranged innervation (1) in Basedow's disease (exophthalmic goitre); (2) in long-continued nervous palpitation from any cause, particularly sexual excesses; (3) certain poisons and articles of diet appear to act in this way, as tea, coffee, alcohol, and tobacco.

In all these cases there is simple over-action or increased functional activity, which, if prolonged, certainly produces some degree of hypertrophy. How this condition is brought about is not very clear. We may suppose the increased frequency of contraction to result from stimulation of the accelerator nerves, as seems probably the case in exophthalmic goitre; from irritability of the cardiac ganglia themselves, owing to the influence of such toxic agents as tea, tobacco, etc.; or from defective vagus control. Long-continued neurotic palpitation in reality causes hypertrophy by increasing the work of the heart, for under perverted stimuli the ventricular contractions are doubled in frequency--sometimes in force as well--while maintaining the circulation in normal vessels offering no increased resistance to the blood-flow.

There can be no doubt of the occurrence of actual hypertrophy as a sequence of the irritable heart induced by sexual excesses and tobacco. I had under observation on and off for several years a very emotional and {620} hypochondriacal young man addicted to venery, whose left ventricle became strongly developed and beat outside the nipple-line. His entire thoughts became centred in his heart trouble, and he travelled from one authority to another in this country and Europe seeking advice.[41] The smoker's heart rarely leads to much hypertrophy, but in young lads it may do so, and even induce more serious disease, as indicated by the presence of murmurs and signs of cardiac failure. The abuse of spirits as a cause of hypertrophy is not very clearly established. Alcoholism appears to be a factor in the production of atheroma. I have been struck by the fact that in four typical instances of so-called idiopathic hypertrophy occurring in powerfully-built workers there was a history of intemperance; and it is quite possible that this may have combined with the muscular efforts in inducing the heart disease; at any rate, it would prove an important element in hastening the final breakdown when from any cause hypertrophy had arisen.

[Footnote 41: After three or four years of most unnecessary worry in the expectation of death from heart disease, this patient has quieted into the belief that there is not anything seriously wrong with his heart, and has now rarely any indications of trouble.]

The majority of cases of hypertrophy of the heart are due to mechanical causes leading to increased resistance and increased work on the part of the organ. Under these circumstances, as in other hollow viscera, the muscle develops, gets thicker and firmer, and capable of accomplishing the extra labor thrown upon it. Defects in the valvular mechanism, obstruction, or incompetency, and increased resistance to the blood-flow in the arteries, are the most important causes of hypertrophy. The ultimate factor in all is heightened pressure within the cardiac cavities due to one of two things--increased volume of blood to be moved or difficulty in propelling the normal volume, caused by obstruction to the flow either central or peripheral.

Pericardial adhesions may impede the action of the heart, and either directly cause hypertrophy or induce dilatation and a consequent hypertrophy.

The details regarding the etiology are best considered in a study of hypertrophy as it affects the individual chambers.

Left Ventricle.--This chamber is much more frequently affected than any other, and may be involved alone or as part of a general enlargement of the organ. The more important causes are as follows:

(1) Aortic Stenosis.--To send the normal charge of blood through a narrowed orifice the muscle must contract with increased force, and to accomplish the work the walls increase in thickness. There may be simple hypertrophy without dilatation of the chamber, but in the later stages this inevitably supervenes.

(2) Aortic Regurgitation.--Curling and foreshortening of the aortic cusps permits of a backward flow into the ventricle during its diastole, with the production of dilatation and increased pressure, to overcome which the walls thicken--eccentric hypertrophy. This is one of the most common causes, and leads to enormous enlargement of the heart.

(3) Mitral Insufficiency.--In extreme grades of mitral stenosis the left ventricle is usually small, but when the curtains are curled and the patent auriculo-ventricular orifice large, there may be very great hypertrophy. Free regurgitation is always accompanied by considerable eccentric hypertrophy, due to the distension of the chamber by the extra quantity of blood forced in at each auricular systole.

(4) Pericardial adhesions, particularly when in addition to union of the layers the parietal membrane is firmly united to the pleura or to the sternum, may cause hypertrophy of the left ventricle alone, but more commonly of the whole heart.

(5) Abnormal Conditions of the Aorta.--(a) Atheroma, with or without dilatation of the arch, is a cause of hypertrophy, for the heart has to {621} compensate for the loss of arterial elasticity, an important factor in the onward movement of the blood during the diastole; and, again, there is increased resistance in the wider tube. (b) Great narrowing, as in the congenital coarctation just beyond the ductus arteriosus, which may produce colossal hypertrophy. Pressure upon the large vessels in the thorax by tumors may act in the same way. (c) Aneurism of the aorta is not often accompanied by hypertrophy unless the valves are affected. Theoretically, it might be expected, as a large saccular dilatation would certainly appear to be a cause of increased resistance, but in uncomplicated cases the experience of most observers appears to accord with that of Stokes,[42] who states that we usually find a small heart. Occasionally, however, there is marked hypertrophy even without valvular disease.

[Footnote 42: Loc. cit.]

(6) Kidney disease, acute and chronic, is very frequently accompanied with hypertrophy of the left ventricle. Indeed, simple hypertrophy is more often met with in chronic Bright's disease than under any other conditions. Increased blood-pressure in the smaller arteries throughout the body is now very generally acknowledged to be the immediate cause. But how this is brought about is a question not yet satisfactorily determined.

We have to deal with two sets of cases. There is the cardiac hypertrophy accompanying acute or subacute nephritis, particularly the scarlatinal. Here there are no chronic arterial changes, and the increased arterial tension appears to be due to contraction of the smaller arteries under the influence of retained excreta, which may act through the vaso-motor centre, as Ludwig observes, or possibly directly upon the unstriped fibres of the tunica media of the arteries. Bright's original explanation still holds good, I think, when he says that the altered quality of the blood "so affects the minute and capillary circulation as to render greater action necessary to send the blood through the distant subdivisions of the vascular system."[43]

[Footnote 43: Guy's Hospital Reports, 1836.]

The hypertrophy of the left ventricle in connection with contracted kidneys is more frequent and more marked. Traube suggested[44] that the interference with the local circulation in the kidneys by the obliteration of vessels increased the work of the heart and induced the hypertrophy, but it is much more probable that the change is a widespread one throughout the body. Gull and Sutton hold[45] that in these cases there is a condition of arterio-capillary fibrosis in which the small arteries are thickened and their calibre diminished, leading in time to a more or less widespread sclerosis in various organs, particularly the kidneys. As a result of this fibrosis, the movement of blood in the smaller vessels is much impeded, the arterial tension increased, and the work of the heart greatly augmented. On the other hand, George Johnson[46] maintains that the muscular coat of the arterioles becomes thickened under the influence of retained excreta, and they are in a state of spasm which increases the tension and heightens the blood-pressure in the left ventricle.

[Footnote 44: Gesammelte Beiträge, Bd. ii.]

[Footnote 45: Medico-Chirurgical Transactions, lv., 1872.]

[Footnote 46: Ibid., vol. xxxiii.]

The question can scarcely be considered settled as regards details, but the general fact of increased peripheral resistance is well established, and it is one of the most frequent causes of non-valvular hypertrophy. It may be quite marked in persons without positive evidence of renal disease as indicated by albumen or casts in the urine, but in whom the condition of arterio-capillary fibrosis is evident from the thickened state of the small arteries, the increased tension, and the firm dislocated impulse of the heart.

(7) Prolonged muscular exertion has been much insisted upon as a cause of cardiac hypertrophy by DaCosta, Myers, Albutt, Seitz,[47] and others. {622} Soldiers, blacksmiths, miners, mountaineers, and men whose occupations call for heavy and prolonged exercise occasionally develop hypertrophy of the heart, which it seems reasonable to connect with the over-use of the muscles. DaCosta's irritable heart in young soldiers appears to represent the early stage of this condition. In 38 per cent. of the cases excessive marching was the cause. He was able to confirm the existence of hypertrophy by autopsy. It is not uncommon to meet with cases of pronounced heart disease, with symptoms of failing compensation, dropsy, etc., in large, powerfully-built men who have been engaged in laborious occupations, and who are admitted to hospital with the clinical picture of chronic valvular disease. At the autopsy one is surprised to find an hypertrophied and dilated heart without valve lesion, perhaps no extensive arterial degeneration, and no kidney disease. They are called cases of idiopathic hypertrophy, but I believe that some of them, at any rate, are instances of a condition induced by prolonged muscular effort. I have had an opportunity of studying carefully four such cases, and I have seen autopsies in two other instances. As I mentioned, alcoholism may be also a factor in these cases, as most of them occur in hard drinkers.

[Footnote 47: Die Ueberanstrengung des Herzens, Berlin, 1875--a collection of six monographs on the subject.]

How muscular effort acts in inducing hypertrophy has been much discussed. It seems rational to suppose that prolonged action of the heart at a rate more vigorous and rapid than normal would induce enlargement of its muscle, just as constant exercise acts with others; and possibly within limits this does take place. Albutt speaks of the large red left ventricles in the Leeds iron-workers killed by accident or cut off by acute disease. No doubt the thickness of the ventricle is measured by the muscular needs of the system. Muscular contraction affects the heart in two ways: first, the venous flow is accelerated, more blood reaches the right heart, and is sent to the lungs, and more reaches the left ventricle and the systemic arteries. The fuller inspirations also favor flow to the heart. When the exercise is excessive the right heart and the venous system become still more distended, and the outflow from the peripheral arteries proportionately retarded and the tension in them increased--particularly is this the case in efforts requiring straining, as in lifting, etc.; and, secondly, the effect of muscular contraction has been shown by Traube to increase very greatly the pressure in the arteries. Gaskell, however, states[48] that when a muscle contracts its own arterioles dilate; but however that may be, the increased tension during muscular contraction can be determined in the radial by the finger, and still better by the sphygmograph, during steady contraction of the muscles of the arm. In yet a third way the blood-pressure may be increased during violent muscular efforts, particularly when the breath is held. The vaso-motor centre is stimulated by the lack of oxygen, and in consequence the blood-pressure rises in the peripheral arteries. At the end of prolonged contests we sometimes see men get pale or the left ventricle may become so embarrassed that they faint.

[Footnote 48: Journal of Physiology, iii.]

(8) That the heart becomes hypertrophied during pregnancy has been specially insisted upon by French writers, Larcher[49] and others. Many doubt the correctness of their deductions, but the weight of evidence seems to point unmistakably to the existence of moderate increase in the thickness of the walls of the left ventricle.[50] Cohnstein[51] connects it with the hydræmic and chlorotic conditions of the blood, so liable to develop during pregnancy.

[Footnote 49: Archives générales, 1859.]

[Footnote 50: McDonald, Heart Disease during Pregnancy, London, 1878.]

[Footnote 51: Virchow's Archiv, lxxvii.]

(9) Hypertrophy of the right heart in disease of the lungs or of the valves is usually followed by more or less hypertrophy of the left ventricle as well, caused by the increased work in consequence of retarded outflow into the venous system.

{623} Right Ventricle.--Hypertrophy of this chamber is most frequently met with in connection with disease of the left side of the heart; next with various chronic affections of the lungs; and lastly with valvular affections of the right side.

(1) Mitral lesions--incompetence or stenosis--are very common causes which act by increasing the resistance in the pulmonary veins and obstructing the free flow of blood in capillaries of the lung. To compensate for this defect the walls of the right ventricle increase in size, and the hypertrophy at first may be unattended with dilatation.

(2) Pulmonary Lesions.--The obliteration of any considerable number of blood-vessels within the lungs by emphysema, cirrhosis, or phthisis (sometimes), occasionally the compression of pleuritic exudation, increases the blood-pressure in the pulmonary artery and rapidly leads to hypertrophy of the right heart. Narrowing of the main branches of the pulmonary artery by the growth of tumors or an aneurism of the aorta occasionally produces the same effect.

(3) Valvular lesions on the right side are rare causes of hypertrophy in the adult, but during foetal life, when endocarditis is more prevalent in the pulmonary and tricuspid valve, stenosis or insufficiency at these orifices leads to great enlargement of the ventricle. Pulmonary stenosis is the most common lesion; incompetence is not often met with. Lesions of the tricuspid valves in the adult are almost always associated with mitral disease. When the dilated hypertrophy of the right ventricle reaches a certain grade in cases of mitral disease or pulmonary lesion, tricuspid incompetence develops.

(4) Among other causes which may be mentioned are pericardial adhesions, which some think tend specially to the production of right-sided hypertrophy and extensive pleuritic adhesions. Atheroma of the pulmonary arteries is more often a consequence than a cause of hypertrophy.

The auricles are usually dilated and hypertrophied; simple hypertrophy is probably never seen. In the left auricle this condition develops in lesions at the mitral orifice, particularly stenosis when it compensates for the obstruction. In free mitral regurgitation the hypertrophy is not so marked.

The right auricle hypertrophies when there is greatly increased blood-pressure in the lesser circulation, whether due to mitral stenosis or pulmonary lesions, and incompetency at the tricuspid orifice. Stenosis of the auriculo-ventricular orifice is a less frequent cause. The dilatation is always excessive.

MORBID ANATOMY.--In general hypertrophy the entire organ is increased in size and weight; more commonly we find the condition limited to two or three chambers or to one side. The estimation of slight grades of enlargement is difficult, but where the increase is marked the process is simple enough. The volume of the heart varies in different individuals according to their age and size. The normal heart is about the size of the closed fist, and, as Virchow suggests, a fair estimate can be made by comparing the two together. By careful weighing we get much more accurate information. The heart of an average-sized man weighs about 9 oz., of a woman about 8 oz. In great hypertrophy the organ may weigh three or four times the normal amount. A heart which weighs over 12 oz. in a man, and over 10 oz. in a woman, may be considered hypertrophied. Hearts weighing from 16 to 20 oz. are not uncommonly met with. Weights above 25 oz. are rare. The heaviest hearts on record are described by Beverley Robinson of New York,[52] 53 oz.; Dulles of Philadelphia, 48 oz.; and there are several cases described in the Transactions of the London Pathological Society of the organ weighing as much as 46 oz.

[Footnote 52: New York Medical Record, 1883.]

{624} Next to weighing, careful measurement of the thickness of the walls is the best means of determining hypertrophy. When there is great dilatation of a chamber the walls, though actually thick, may look proportionately thin; and on the other hand, when rigor mortis is present the cavity may be very small and the walls appear enormously thick. In this case measurements should not be made until the heart has been soaked in water and thoroughly relaxed. The normal thickness of the left ventricle is about half an inch (12 or 13 millimeters), being thicker toward the base. It is well to measure in two or three places, not including the papillary muscles. A thickness of 10 lines or over (20 to 25 mm.) indicates hypertrophy. It is rare to meet with the wall thicker than 1 inch (25 mm.), even in very great hypertrophy. The right ventricle is thinner than the left, and has an average diameter of from 2 to 3 lines (4 to 7 mm.). A thickness of from 6 to 9 lines (13 to 20 mm.) may be met with in great hypertrophy. It is very rare to see a diameter of more than three-quarters of an inch, but cases are reported of a thickness of over an inch. The left auricle has a normal thickness of about a line and a half (3 mm.), which in considerable hypertrophy may be nearly doubled. The wall of the right auricle is even thinner than the left, rarely exceeding 1 line in diameter. In hypertrophy the sinus does not present a marked increase in thickness, but the appendix, particularly the musculi pectinati, may be greatly developed and measure from 2 to 3 lines in diameter.

The shape of the heart is much affected by the degree of hypertrophy in different cavities. Great enlargement of the ventricles broadens the apex, and the conical shape is lost. In the enormous hypertrophy and dilatation of aortic insufficiency the increased breadth and rotundity of the apex becomes very marked. When the right ventricle is chiefly affected, it occupies a large share of the apex, and the transverse diameter of the organ is increased. When due to mitral stenosis the contrast between the large broad right ventricle extending well to the apex and the small left chamber is very striking.

When not degenerated the muscle-tissue of an hypertrophied heart is of a deep-red color, firm, and usually cuts with slightly increased resistance. The right ventricle often has a peculiarly hard, leathery feel, which was noticed by Rokitansky. In simple hypertrophy of the left ventricle the papillary muscles and columnæ carneæ may be increased in size, but the former often appear flattened in great eccentric enlargement. The trabeculæ are usually much more developed in the right ventricle and in the appendix of the right auricle than in the left chambers. Very often the tissue looks pale, and may be soft from the occurrence of fatty degeneration.

The histological characters of the changes in hypertrophy have been much studied, particularly with a view of determining the question of numerical increase. Hepp[53] described an increase in the thickness; but most recent observers regard the hypertrophy as due to numerical increase, resulting from the development of new fibres, either by the splitting of the old ones (Rindfleisch) or their growth from interfibrillar nuclei.[54] Wilks and Moxon[55] and Gowers[56] find that the fibres are not increased in size. Letulle[57] thinks that there is a process of progressive hyper-nutrition of the fibres.

[Footnote 53: Henle's Zeitschrift, 1854.]

[Footnote 54: Zielonko, Virchow's Archiv, lxii.]

[Footnote 55: Pathological Anatomy, London, 1875.]

[Footnote 56: Reynolds's System.]

[Footnote 57: Quoted by Peter, loc. cit., p. 280.]

The toughness of the hypertrophied muscle is due to the increase in the connective tissue, which is more marked as a rule in the right than the left ventricle. Sometimes, indeed, it is not at all noticeable in the latter, which may be soft and tears readily with the finger.

SYMPTOMS.--Hypertrophy is a conservative process, usually secondary to some valvular or arterial lesion, and is not necessarily accompanied by any {625} symptoms. So admirable is the adjusting power of the heart that, for example, an advancing stenosis of aortic or mitral orifice may be for years perfectly counterbalanced by a progressive hypertrophy, and the subject of the affection be happily oblivious to the existence of heart trouble. Particularly is this the case with mitral stenosis and the consequent hypertrophy of the left auricle and right ventricle. While leading quiet lives and not straining the heart with violent exertion, such persons may not suffer in any way, or perhaps only experience a little shortness of breath when going up stairs. Indeed, the hypertrophy is in almost all instances an unmixed good, and many of the symptoms which arise are to be attributed to its failure, or, as we say, disturbance of compensation.

The left ventricle is most often involved, and the clinical features of hypertrophy are best seen when it is affected. Inspection may reveal decided bulging of the præcordia, producing in extreme instances marked asymmetry of the chest. This is most frequent in persons under twenty years of age, and it may occur without any pericardial adhesions, which Shroetter[58] thinks are invariably associated with this condition. The intercostal spaces may be widened, and the area of visible impulse is much increased. On palpation the character and position of the apex-beat give most important results. It is stronger, more forcible and heaving, and may lift the chest-wall. With each systole the hand or the ear applied over the heart may be visibly raised. A slow heaving impulse is one of the best signs of simple hypertrophy; when there is large dilated hypertrophy the forcible impulse is often more sudden and abrupt. A second, weaker, impulse can sometimes be felt, due possibly (as Gowers suggests) to a rebound from the aortic valves. The area of impulse is greatly increased, and the beat may be felt in the sixth, seventh, or eighth interspace from an inch to three inches outside the nipple. The downward dislocation of the apex is an important sign in hypertrophy of the left ventricle; simple outward displacement may be due to enlargement of the right ventricle.

[Footnote 58: Ziemssen's Encyclopædia, vol. vi.]

In moderate grades of hypertrophy, as seen in chronic Bright's disease, the apex-beat may be in the sixth interspace in the nipple-line or a little outside it.

Percussion gives an area of increased dulness, due to the much larger portion of the heart which comes in contact with the chest-wall. The dulness in the parasternal line may begin at the third rib or in the second interspace, and the transverse limits extend from half an inch to two inches beyond the nipple-line, and an equal distance beyond the middle line of the sternum. The dull region is more ovoid than in health. When carefully delimited and measured, there may be in the colossal hypertrophy of aortic valve disease an area of dulness from seven to eight inches in transverse extent. In moderate grades a transverse dulness of four inches is not uncommon.

On auscultation the heart sounds, when there is no valve disease, may not present any special changes, but the first is often prolonged and dull; but when there is dilatation as well, it may be very clear and sharp. Reduplication is not uncommon, particularly in the hypertrophy of renal disease. A peculiar clink--the tintement métallique of Bouillaud--may sometimes be heard, with the impulse most frequently just to the right of the apex-beat. The second sound is clear and loud, sometimes ringing in character or reduplicated. When the hypertrophy depends upon valvular lesions the sounds are of course much altered, and replaced or accompanied by murmurs.

The pulse of simple hypertrophy not dependent on valvular lesions is usually firm, full, and strong, of high tension, and regular. It may be increased {626} in frequency, but often is normal. In eccentric hypertrophy the pulse is full, but softer, and usually more rapid. So long as the hypertrophy is maintained the pulse is regular; one of the earliest signs of failure and dilatation is irregularity and intermittence. The various modifications of the pulse in connection with valve disease are considered elsewhere.

Among symptoms which patients complain of most frequently are unpleasant sensations about the heart--a sense of fulness and discomfort, rarely amounting to pain. This may be very noticeable when recumbent and on the left side. Actual pain in simple hypertrophy is rare, but in the irritable heart from tobacco and in neurasthenics with slight enlargement it is often a very troublesome symptom. Palpitation is not often complained of, nor do patients always have sensations from the violent shocks of a greatly hypertrophied organ; others, again, will have very uneasy feelings from a moderately exaggerated pulsation. The general condition of health has much to do with this: we are not in health conscious of our own heart's action, but one of the very first indications of nervous exhaustion from excesses or over-study is the consciousness of the heart's action, not necessarily accompanied by palpitation. Flushings of the face, noises in the ear, flashes of light, and headaches are not uncommon.

There are certain untoward effects of long-continued hypertrophy of the left ventricle which must be mentioned, chief among which is the production of atheromatous degeneration of the vessels. Particularly is this the case when the hypertrophy results from increased peripheral resistance. The heightened blood-pressure in the arteries (which is expressed by the word strain) gradually induces an endarteritis and a stiff, inelastic state of those vessels most exposed to it--viz. the aorta and its primary divisions. In overcoming the peripheral obstruction the hypertrophy "ruins the arteries as a sequential result" (Fothergill). It is in this way that prolonged muscular exertion acts injuriously, and leads to two common morbid conditions in athletes and persons whose employment necessitates violent exercise of the muscles--viz. aneurism and sclerosis of the aortic semi-lunar valves, with incompetency. Syphilis certainly does not embrace the entire etiology of aneurism, the occurrence of which in soldiers, strikers, foundrymen, etc. can be traced to arterial strain. So also with the sclerosis of the semi-lunar valves--just enough, perhaps, to produce incompetency; how common it seems to be in strong, well-built men whose excesses have been on the cinder-path or on the river! The increased aortic tension, with the more forcible recoil and closure of the semi-lunar valves, would seem to be factors in the production of this condition. Aortic incompetency is the special danger of athletes, and no inconsiderable number of the cases of this lesion occurring in men without rheumatic or syphilitic history may be traced to over-use of the muscles.

Another special danger is rupture of the blood-vessels, particularly of the brain. In the condition of general arterial degeneration associated with contracted kidneys and hypertrophied left heart apoplexy is common; indeed, we may say that in the majority of cases of cerebral hemorrhage there is sclerosis of the cerebral vessels, often with the development of miliary aneurisms, and the rupture is directly induced by the forcible action of the heart.

Hypertrophy of the right ventricle in the adult is rarely induced by valvular disease on the right side, but is a result of increased resistance in the pulmonary circulation, as in cirrhosis of the lung and emphysema, or in stenosis of the mitral orifice. When the compensation is perfect, and the hypertrophy fully maintains the equilibrium of the circulation, there are no symptoms. Extra exertion, as in ascending stairs or running, may induce shortness of breath, but in many respects hypertrophy of {627} the right ventricle is the most enduring and salutary form in the whole range of cardiac affections. For long periods of years the effects of mitral stenosis may be counterbalanced completely, and only sudden death by accident or an acute disease reveals the existence of extensive unsuspected heart disease. In the hypertrophy secondary to pulmonary disease, particularly emphysema and cirrhosis, there may be sensations of uneasiness in the cardiac region, with cough and shortness of breath; but so long as the dilatation is moderate the symptoms are not marked. With great dilatation and tricuspid regurgitation come the venous engorgement, oedema, and pulmonary troubles. The increased pressure in the lesser circulation not uncommonly leads to atheroma of the pulmonary artery, and the full state of the capillaries leads ultimately to a deposition of pigment and increase in the fibrous elements in the lung--the brown induration. Pulmonary congestion and apoplexy from thrombosis or embolism are more often associated with dilatation. Hæmoptysis may result from rupture of vessels during sudden exertion.

The physical signs of hypertrophy of the right ventricle are not so marked as those of the left. Bulging of the lower part of the sternum and left cartilages is occasionally met with. The apex-beat is forced to the left, but is not so often displaced downward. The most marked impulse may be in the epigastrium, in the angle between the ensiform cartilage and the seventh rib or beneath the cartilages of the sixth and seventh ribs. The pulsation is rarely the strong heave of left-sided hypertrophy, and is apt to be diffuse, not punctuate, particularly if there is much dilatation. In thin-walled chests there may be pulsation in the third and fourth right interspaces. The area of dulness is increased in the transverse direction, particularly toward the right, where it may extend an inch or more beyond the border of the sternum. On auscultation the first sound at the lower part of the sternum is louder and fuller than normal, but the differences are not very marked unless there is much dilatation, when it is clearer and sharper. The second sound is accentuated in the pulmonary artery on account of the increased tension, and there may be reduplication. The pulse at the wrist is usually small. The jugular pulsation occurs when there is tricuspid incompetence, which arises when the eccentric hypertrophy reaches a certain grade.

Hypertrophy of the auricles is always associated with dilatation. It is most common in the left chamber, which hypertrophies in mitral stenosis and incompetency, and assists materially in restoring the balance of the circulation and protects the lungs. There are no special physical signs, and we usually can only infer its presence by the existence of mitral stenosis and a presystolic murmur. Increased dulness may be determined at the left of the sternum, and there may be a presystolic wave in the second left interspace.

Hypertrophy and dilatation of the right auricle occur not infrequently, and are almost invariably associated with a similar condition in the right ventricle, and incompetency of the tricuspid. In emphysema, cirrhosis of the lung, chronic bronchitis, and in mitral disease, it is very common, much more so than the statement of some authors would lead us to expect. In comparison with the left auricle the greater development and hypertrophy of the appendix and its musculi pectinati is very striking. The latter may be distributed over the anterior wall of the sinus to a much greater extent than in health. There may be increased dulness in the third and fourth interspaces, with pulsation presystolic in rhythm. Usually there are signs of venous engorgement, jugular pulsation, and other evidences of dilatation of the right heart.

The DIAGNOSIS of cardiac hypertrophy does not usually present any serious difficulties. Increase in size, more forcible contraction, with displacement of {628} the apex-beat, and the character of the pulse, are the most important signs. There are certain conditions which require to be carefully distinguished. Neurotic palpitation, from whatever cause, may be accompanied with forcible contraction, but it has not the heaving impulse of genuine hypertrophy. Actual enlargement of the organ may, however, result from prolonged over-action, as in Basedow's disease, in the smoker's heart, and the irritable heart of neurasthenics, but it is usually slight. Increased dulness in the cardiac area may be due to a variety of causes, some of which may simulate hypertrophy, as pericardial effusion, aneurism, mediastinal growths, or displacement of the heart from pressure or the existence of malformation of the chest; but with the exercise of ordinary care the diagnosis can usually be made. There are two opposite conditions which not infrequently give trouble. When the left lung is retracted from pleurisy, phthisis, or cirrhosis, there is a large surface of the heart exposed, and the pulsation may be extensive and forcible, and at first sight resemble hypertrophy. There is usually in this condition some dislocation upward and to the left. The history of pulmonary or pleuritic disease, and the evident fixture of the lung on deep inspiration, will usually suffice to prevent mistake. A similar exposure of the heart occurs without any disease in very narrow-chested persons with ill-developed lungs; and here, though the area of dulness may be much increased, yet the normal position of the apex and the absence of forcible heaving impulse, pulse signs, and of any obvious cause of hypertrophy will afford satisfactory criteria for a diagnosis. Just the reverse occurs in some cases in which a moderate cardiac hypertrophy is masked by emphysema of the lungs or of their anterior borders. The area of dulness may be normal, or even diminished, and the pulsation diffuse and chiefly epigastric. The general condition, state of the pulse, and character of the sounds would help in the diagnosis, but it is sometimes a matter of no little difficulty.

The symptoms and physical signs above narrated sufficiently indicate the points of difference between hypertrophy of the two sides of the heart.

In all cases the greatest possible care should be exercised in ascertaining the presence or absence of conditions likely to cause hypertrophy.

The COURSE of a case of ordinary hypertrophy may be divided into three stages: 1st. The period of development, which varies much with the nature of the primary disease. Thus in rupture of an aortic cusp or in sudden overstrain from exertion it may require months, or even years, before the hypertrophy becomes fully developed. In these cases it may never do so, and then death results. On the other hand, in sclerotic affections of the valves with stenosis or incompetence the hypertrophy develops pari passu with the lesion, and may continue to counterbalance a progressive impairment of the valves. 2d. The period of full compensation, the latent stage, during which the heart's vigor meets all the requirements of the circulation. There may be no signs whatever of heart weakness, but the hypertrophied muscle completely equalizes the valvular or other defects. It may last an indefinite period of years. In some cases this fortunate period is never fully attained, and indications of incomplete compensation remind the individual that he has a heart affection. 3d. The period of disturbed compensation, which sooner or later awaits all victims of hypertrophy. It may come suddenly during an extra exertion, and death follow from acute dilatation; or more commonly it takes place slowly, and results from degeneration and weakening of the heart-muscle, with consequent dilatation and all its evils. There may be repeated failures before the end is reached, represented clinically by attacks of cardiac dyspnoea and dropsy.

The breaking, as it is called, of a compensatory hypertrophy may be induced by many causes. Among the most important is failure of general or local (cardiac) nutrition. In many a chronic heart case readmitted to {629} hospital, perhaps for the third or fourth time, with dyspnoea and dropsy, exposure, poor food, and whiskey are responsible for the failure. Gradual sclerosis of the coronary arteries leading to fatty or fibroid changes is a fruitful source of disturbed compensation. It is well known that during or after an acute illness, pneumonia, fever, or a bronchial attack the first symptoms of heart disease may be manifested. Mental emotions, severe grief, or fright have been known to bring on symptoms of heart failure in hypertrophy. One of the most frequent causes is sudden or prolonged muscular exertion, which may disturb a compensation perfect for years, and induce death in a few days.[59] The intimate pathology of broken compensation is not always clear. It certainly does not always depend on degeneration of the muscle-fibres, so far as microscopical examination can tell, and in many cases we are forced to conclude that the ganglia are at fault and the breakdown is nervous, not muscular.

[Footnote 59: Traube, Gesammelte Beiträge, Bd. iii.]

The PROGNOSIS depends entirely upon the nature of the cause which has induced the hypertrophy. When remediable or removable, the heart may return to its normal size, as after pregnancy, acute Bright's disease, and some cases of hypertrophy from deranged innervation and muscular exertion. When the cause is irremediable, as in chronic valve disease, sclerosis of the arteries, or obliteration of pulmonary capillaries, the case is quite different. Here the prognosis depends largely on the capability of maintaining in its integrity a sufficient hypertrophy to compensate for the obstruction: so long as this keeps up all is well; the evils come with failure of the hypertrophy and increase of the dilatation. Conditions of general and local nutrition are all-important factors, and when these can be supported to the highest possible degree the prognosis is favorable. Ill-health may be indicated at once by the onset of cardiac symptoms, pointing to disturbed compensation. Much depends on the seat of the original disease. Mitral stenosis carries with it as good prognosis, quo ad longevity, as aortic stenosis,[60] and the latter much better than aortic insufficiency. The nutrition of the muscle of the heart demands a full and constant supply of blood, but in aortic incompetency the rapid regurgitation does not permit of the complete distension of the coronary vessels,[61] and the strain is such that atheroma of these arteries is very apt to follow and still further diminish the blood-supply. Hence the prognosis in aortic insufficiency for enduring hypertrophy is bad. The hypertrophy which accompanies general arterial degeneration, though compensating for peripheral obstruction, carries with it certain dangers, as already {630} indicated, in the liability to cause rupture. With care such patients may survive for years, though exposed to risks other than cardiac.

[Footnote 60: Brückes held that the coronary vessels were filled in diastole alone, but there can be no question that blood also enters during the systole. The sigmoid valves certainly do not in the majority of cases cover the orifices of these arteries during this act. Undoubtedly, however, the heart-vessels are more distended in diastole. The pallor of the muscle in systole is a proof that the coronary vessels are not well filled at this period.]

[Footnote 61: This is not the usual statement, but my experience--limited, it is true--seems to point to the conclusion that mitral stenosis may also exist for many years without exciting symptoms of heart disease. It may, I think, be safely affirmed that a larger number of persons with mitral valve disease live in blissful ignorance of the existence of serious heart lesion than any other group of cardiac cases. Particularly is this the case in women. Two points have attracted my attention in this connection: the frequency with which we find evidence of stenosis--as shown by the presystolic thrill and rough murmur--in women complaining, perhaps, of shortness of breath on exertion and slight cardiac distress--symptoms which are readily relieved--and the discovery post-mortem of stenosis of the mitral orifice in cases of sudden death by embolism or from some intercurrent disease occurring in persons in whom heart disease had never been suspected. The narrowing may be extreme--an orifice only 13 millimeters in width in one case in which a woman was stricken with hemiplegia while attending to her household duties. Such cases, and they are not very uncommon, teach us how perfect compensation may be in this lesion.]

The TREATMENT of hypertrophy consists largely of measures directed toward its maintenance in a degree proportionate to the extra work which the heart has to do. In organic disease the well-being of the patient depends on this: we cannot remove the cause, but we can by careful hygienic and dietetic regulations maintain the balance between the defect and the compensation. The original lesion is usually beyond control, and the special indications are to moderate certain dangers associated with hypertrophy, and to promptly meet the earliest symptoms of heart failure. The utmost moderation in food, drink, and exercise must be enjoined. Quiet, regular habits are all important; excesses of all kind quickly lead to impairment of the heart's action. In the hypertrophy associated with arterial and renal disease a special danger exists in the tendency to rupture of vessels. In these cases vigorous heart-beat, with very high tension in the peripheral arteries, indicates mischief which may be met by taking prompt measures for the reduction of the high pressure. A brisk cathartic may avert an apoplectic attack, and there are cases in which the old practice of bleeding--formerly so much in vogue for hypertrophy--is justifiable. Palpitation and shortness of breath are among the earliest signs of failing compensation, and call for the treatment to be considered under Dilatation. The condition of hypertrophy from organic disease is not directly amenable to treatment; we cannot diminish the size of the organ, but we can regulate its action by measures which control the contractions when from any cause they become too forcible or irregular. More particularly is this the case in hypertrophy due to disturbed innervation. When vigorous, rest and the administration of cardiac sedatives, such as aconite or veratrum viride, will generally suffice to reduce the force of the contractions. The palpitation and irregular action in cases of irritable heart from over-exertion, the abuse of tobacco, or sexual excesses may subside with the removal of the cause. The steadying action of small doses of digitalis is often well seen in these cases.

Dilatation of the Heart.

DEFINITION.--An increase in the size of one or more of the chambers, with or without thickening of the walls.

VARIETIES.--Two varieties may be recognized: (1) dilatation with thickening, and (2) dilatation with thinning. Dilatation with thickening is the most common, and corresponds with the dilated or eccentric hypertrophy and the active dilatation of some writers. Those cases of dilatation with walls of apparently normal thickness--simple dilatation of authors--also belong to this category, for if the chamber is distended, and yet the walls maintain their normal diameter, they must of course be hypertrophied. The dilatation with thinning--passive dilatation--is specially met with in the auricles, and is characterized by increase in the size of the chamber and attenuation of the walls.

The diastole of the heart is partly an active, partly a passive act. The cavities behave as would rubber balls, and their distension after contraction is partly due to their elasticity. The heart is a suction- as well as a force-pump. In the ventricles, for example, after systole the active dilatation draws blood from the auricle--must do so, in fact, in the very process of dilating--and then the auricular systole completes the process, fully accomplishing the diastole. Dilatation occurs during this period, and results from distension beyond the limits of the contractile power of the wall. More blood is contained in the cavity than the muscle of the wall can {631} control--i.e. expel--but if the organ is healthy, hypertrophy ensues and the chamber accommodates itself to the altered condition. It is the heightened pressure during diastole which is dangerous; during systole the pressure may be extreme, and yet no dilatation may ensue, as in aortic stenosis, in which condition the size of the chamber may remain normal, and yet the walls hypertrophy to meet the greatly-increased resistance to the outflow of the blood during the systole. In the auricles, however, the increased tension during contraction may be accompanied with considerable dilatation, as in mitral stenosis.

ETIOLOGY.--There are two important causes in the production of dilatation: increased pressure within the cavities, and impaired resistance due to disease of the muscular substance of the heart. They may act singly, but are often combined. Weakened walls may yield under normal distending force, or normal walls may yield under a heightened blood-pressure, or both factors may prevail.

1. Increased endocardiac pressure--which results, as before stated, either from an augmented quantity of blood to be moved or an obstacle to be overcome--is the most frequent cause of dilatation. It does not necessarily cause it. Simple hypertrophy may be the result, as in the early period of aortic stenosis and in the hypertrophy of the left ventricle in Bright's disease.

Most of the important causes of increased endocardiac pressure have already been considered under Hypertrophy, but we may refer to one or two more particularly.

The size of the cardiac chambers is variable in conditions of health. With slow action of the heart the dilatation during diastole must be much more full and complete than with rapid action. Physiologically, the limits of dilatation have been reached when the chamber cannot be emptied during the systole. We find this as an acute, transient condition in severe exertion--during, for example, the ascent of a steep mountain. There may be great distension of the right heart, as shown by the increased epigastric pulsation, and even increase in the cardiac dulness. The safety-valve action of the tricuspid valves may here come into play, and by permitting regurgitation into the auricle relieve the lungs. Rest causes it to pass off, but if it has been extreme, the heart may suffer a strain from which it may recover slowly, or, indeed, the person may never again be able to undertake severe exertion. In the process of training the getting wind, as it is called, is largely a gradual increase in the capability of the heart, particularly the right chambers. A degree of exertion can be safely maintained in full training which would be quite impossible under other circumstances, because by a gradual process of what we may call physical education the heart has strengthened its reserve force--widened enormously its limits of physiological work. Endurance in prolonged contests is measured by the capabilities of the heart, and its essence consists in being able to meet the continuous tendency to overstep the limit of dilatation.

We have no definite information as to the nature of the change in the heart which occurs in the process of training, but it must be in the direction of increased vigor, muscular and nervous. The large hearts often noted in athletes may be due, as already mentioned, to the prolonged use of their muscles; but probably no one can become a great runner or oarsman who has not naturally a large and capable heart. Master McGrath, the celebrated greyhound, and Eclipse, the race-horse, both famous for endurance rather than speed, had very large hearts.

Over-training and heart-strain are closely connected with this question of excessive dilatation during severe muscular effort. Both mean the same thing in many cases. A man, perhaps not in very good condition, calls upon his heart for much extra work during a race or the ascent of a very steep {632} mountain, and is seized with cardiac pain and a feeling of distension in the epigastrium, and the rapid breathing continues an unusual time, but the symptoms pass off after a night's quiet. An attempt to repeat the exercise is followed by another attack, or indeed an attack of cardiac dyspnoea may come on while he is at rest.[62] For months such a man may be unfitted for severe exertion, or may be permanently incapacitated. He has overstrained his heart and has become broken-winded. We see the same thing sometimes in horses. What exactly has taken place in these hearts we cannot say, but their reserve force is lost, and with it the power of meeting the demands exacted in maintaining the circulation during severe exertion.[63] The heart-shock of Latham[64] includes cases of this nature--sudden cardiac breakdown during exertion and not due to rupture of a valve. It seems probable that some cases of sudden death in men and animals during long-continued violent efforts, as in a race, are due to over-distension and paralysis of the heart.

[Footnote 62: In St. George's Hospital Reports, 1872, Clifford Albutt gives his own experience.]

[Footnote 63: H. C. Wood tells me he believes that wind in athletes is in large part a question of vagus control, and that he has noticed in races of dogs used in hunting and other violent exercise the vagi are more sensitive and powerful than in sedentary breeds. He thinks that a similar difference exists between tame and wild rabbits.]

[Footnote 64: Diseases of the Heart, New Sydenham Soc. ed.]

In the various forms of valvular disease we meet with numerous examples of dilatation. In aortic incompetency during diastole blood enters the left ventricle from the unguarded aorta and from the left auricle, and the amount of blood at the termination of diastole subjects the walls to an extreme degree of pressure, under which they inevitably yield: in time they augment in thickness, and we have the typical eccentric hypertrophy of this condition.

In mitral regurgitation a certain quantity of the blood which should have been driven into the aorta is forced into the auricle from which it came, dilating it; and then in the diastole of the ventricle a larger amount is returned from the auricle, and with increased force, by the hypertrophied walls of this chamber. In mitral stenosis the left auricle is the seat of greatly-increased tension during systole, and dilates as well as hypertrophies; the distension too may be enormous. Dilatation of the right chamber is very common, and is produced by a number of conditions, which were considered under Hypertrophy. All circumstances which permanently increase the tension of the blood in the pulmonary vessels will cause it--mitral stenosis, emphysema, etc. The dilatation seems easily produced, but the accompanying hypertrophy may hold it in check for years. We may here refer to the extreme distension of the right chambers in pneumonia, particularly when the consolidation is extensive. The passive dilatation may be very great and the walls much thinned, and we see the same in states of asphyxia. Valvular lesions of the right heart are not frequent causes of dilatation. When the causes which bring about the dilatation act suddenly, the degree of distension may be great, and there is much more difficulty in the establishment of compensation, as in rupture of an aortic cusp.

2. Impaired nutrition of the heart-walls from degeneration or inflammation may lead to such a diminution of the resisting power that dilatation readily occurs.

In fevers the loss of tone due to parenchymatous degeneration or myocarditis may lead to a condition of acute dilatation which may prove fatal. It is a well-recognized cause of death in scarlatinal dropsy,[65] and may occur in rheumatic fever,[66] typhus, typhoid, erysipelas, etc. The myocarditis accompanying acute endo- or pericarditis may lead to dilatation, especially in the latter disease. The cavities are usually large in fatty degeneration or {633} infiltration from the relaxed and atonic state of the walls. In anæmia, leukæmia, and chlorosis the dilatation of the chambers may be considerable. In fibroid degeneration the wall generally yields where the process is most advanced, as at the left apex. The impaired nutrition in coronary disease may lead to dilatation. Under any of these circumstances the walls may yield with normal blood-pressure, or if increased tension is present the effect is the more readily produced.

[Footnote 65: Goodhart, Guy's Hospital Reports, Series iii. vol. xxiv.]

[Footnote 66: Samuel West, Barth. Hospital Reports, xiv.]

Pericardial adhesions are usually spoken of as a cause of dilatation, acting by traction from without, and we generally find in a case of extensive and firm union considerable hypertrophy and dilatation. In this condition there is usually some impairment of the superficial layer of muscle which may permit of over-distension.

MORBID ANATOMY.--Usually the condition exists in two or more chambers, and is associated with hypertrophy, the appearances of which have already been described. It is more common on the right side than on the left. Perhaps the most general dilatation which we see is in cases of aortic incompetency, in which all the cavities may be enormously distended. In mitral stenosis the left auricle is often trebled in capacity, and the right auricle and ventricle also are very capacious. The former may contain eighteen to twenty ounces of blood. In many chronic affections of the lungs the right chambers are chiefly affected. Dilatation with thinning is often the result of an acute process met with in the fevers. The walls may be very much thinner than normal, almost membranous, and the dark color of the blood may show through with distinctness. When the distension of one ventricle is very great, there may be a distinct bulging of the septum toward the other side. The shape of the organ is altered, and when the right chambers are chiefly affected it is more globular in shape. Distension of the left auricle may render it visible in the front of the heart, and the appendix may be prominent. The right auricle when enormously enlarged, as in some cases of pneumonia, in emphysema, and in leukæmia, may form a large mass occupying a considerable space in the antero-lateral part of the thorax. The walls in dilatation with thinning are flabby and relaxed, and collapse at once when cut, but in dilatation with hypertrophy they are firm, especially those of the right ventricle.

The auriculo-ventricular rings are often dilated, and there may be an inch and a half, or even two inches, of increase in the circumference. Thus, the tricuspid orifice, the circumference of which is about four and a half inches, may admit freely a graduated heart-cone of over six inches, and the mitral orifice, which is about three and a half inches normally, may admit the cone to five and a half inches or even more. Great dilatation is always accompanied with relative incompetence of the valves, so that free regurgitation into the auricles is permitted. The orifices of the cavæ and of the pulmonary veins may be greatly dilated.

The muscle-substance varies much in appearance according to the presence or absence of degenerations. The endocardium is often opaque, particularly in the auricles. The microscopical examination may show marked fatty or parenchymatous change, but in other instances of dilatation and heart failure in eccentric hypertrophy there may be no special alteration noticeable. I fully agree with Niemeyer's assertion, "that it is not possible by means of the microscope to recognize all the alterations of the muscular fibrillæ which diminish the functional power of the heart."[67] We know too little as yet of the changes in the ganglia of the heart in these conditions: as centres of control they probably have more to do with cardiac atony and breakdown than we generally admit. Degeneration of them has been noted by Putjakin[68] and others.

[Footnote 67: Textbook of Medicine, vol. i., Am. ed.]

[Footnote 68: Virchow's Archiv, lxxiv.]

{634} SYMPTOMS AND PHYSICAL SIGNS.--Dilatation produces weakness of the cardiac walls, diminishes the vigor of their contractions, and is thus the very reverse of hypertrophy. So long as compensation is maintained the enlargement of a cavity may be considerable: the limit is reached when the hypertrophied walls can no longer in the systole expel all the contents, part of which remain, so that at each diastole the chamber is abnormally full. Thus in aortic incompetency blood enters the left ventricle from the aorta as well as the auricle, dilatation ensues, and also hypertrophy as a direct effect of the increased pressure and increased amount of blood to move. But if from any cause the hypertrophy weakens, and the ventricle during systole does not empty itself completely, a still larger amount is in it at the end of each diastole, and the dilatation becomes greater. The amount remaining after systole is a cause of obstruction, preventing the blood entering freely from the auricle. Incompetency of the auriculo-ventricular valves follows with dilatation of the auricle and impeded blood-flow in the pulmonary veins. Dilatation and hypertrophy of the right heart may compensate for a time, but when this fails stasis occurs in the venous system, with dropsy. The consideration of the symptoms of chronic valvular lesions is largely that of dilatation and its effects. Acute dilatation, such as we see in fevers or in sudden failure of an hypertrophied heart, is accompanied by three chief symptoms--weak usually rapid impulse, dyspnoea, and signs of obstructed venous circulation. Cardiac pain may be present, but it is often absent.

The physical signs of dilatation are those of a weak and enlarged organ. The impulse is diffuse, often undulatory, and is felt over a wide area, and an apex-beat or a point of maximum intensity may not exist. When it does it may be visible, and yet cannot be felt--an observation of Walshe's which is very valuable. An extensive area of impulse with a quick, weak maximum apex-beat may be present. When the right heart is chiefly dilated the left may be pushed over so as to occupy a much less extensive area in the front of the heart, and the true apex-beat is not felt; but the chief impulse is just below or to the right of the xiphoid cartilage, and there is a wavy pulsation in the fourth, fifth, and sixth interspaces to the left of the sternum. In extreme dilatation of the right auricle a pulsation can sometimes be seen in the third right interspace close to the sternum, and with free tricuspid regurgitation this may be systolic in character. Whether the pulsation frequently seen in the second left interspace is ever due to a dilated left auricle is not satisfactorily determined. I have sometimes thought it was presystolic in rhythm, though it may be distinctly systolic. Post-mortem, it is rare in the most extreme distension to see the auricular appendix so far forward as to warrant the belief that it could beat against the second interspace. The area of dulness is increased, but an emphysematous lung or the full distended organ in a state of brown induration may cover over the heart and limit greatly the extent. The directions of increase were considered when speaking of Hypertrophy with dilatation.

The first sound is shorter, sharper, and more valvular in character, and more like the second. As the dilatation becomes excessive it gets weaker. Reduplication is not common, but occasionally differences may be heard in the joint sound over the right and left hearts. Murmurs very frequently obscure the sounds; they are produced by incompetency of the valves due to the great dilatation, or are associated with the chronic valve disease on which the condition depends. The aortic second sound is replaced by a murmur in aortic regurgitation; the pulmonary is accentuated in mitral regurgitation and pulmonary congestion, but with extreme dilatation it may be much weakened. The heart's action is irregular and intermittent, and the pulse is small, weak, and quick.

The DIAGNOSIS is generally easy when the physical signs, the history, and {635} the general condition are taken into account. In a case of valvular disease with hypertrophy the onset of dyspnoea and venous stasis with dropsy tell unmistakably of cardiac dilatation. Increased præcordial dulness, with a weak, diffuse impulse, is not simulated by many conditions, and one only, pericardial effusion, need be specially mentioned. This may present very serious difficulties, and indeed a dilated heart has been aspirated under the belief that effusion was present. The points to be attended to are--the greater lateral dulness in dilatation and the wavy impulse which may extend over a great part of it; in effusion the dulness extends upward and is more pear-shaped, the impulse is not so extensive, and may be tilted up an interspace or may not be visible. The sounds in pericardial effusion are muffled and distant over the dull region, but at its upper limit may be clear. The absence of friction is an important negative sign. In some cases it is extremely difficult to determine between the conditions, and I have known a weak, feeble, irregular heart, with cyanosis, and oedema lead to the diagnosis of dilatation when effusion was present.

The PROGNOSIS depends upon the cause of the dilatation. In anæmia and fevers the temporary dilatation may undoubtedly pass away with the improvement of health; but when the cause is not remediable the danger must be measured by the presence or absence of compensation. In the majority of the cases which we see the dilatation occurs in valve disease, and no symptoms of importance arise so long as the compensation is perfect. Failure of this, which may result from many causes, as already mentioned, is always serious. It may be only temporary, and with care the compensation can be re-established and the symptoms pass away. We constantly see this in the eccentric hypertrophy of the right heart from mitral disease; an attack of bronchitis suffices to disturb the compensation, and with the relief of the catarrhal trouble the dyspnoea and heart symptoms disappear.

The TREATMENT of dilatation is virtually that of chronic valvular disease, and we shall only refer to general indications. With the earliest symptoms of failure the work of the heart should be reduced to a minimum by placing the patient at rest. This in itself may suffice without any other measures. Time and again I have seen, particularly in cases of aortic insufficiency, the dyspnoea relieved and the oedema of the feet disappear and the compensation re-established by placing the patient in bed, enjoining absolute quiet and carefully regulating the diet. The importance of rest in the early stages of heart failure cannot be too much insisted upon.[69] Quiet and careful dieting may suffice for the milder attacks, but we have usually even in these to resort to heart tonics. Digitalis is the most powerful remedy we possess in restoring and maintaining compensation. Under its use the irregular, feeble, and frequent contraction becomes regular and stronger, and the embarrassed circulation is relieved. In hospital practice the same chronic heart cases may return year after year with attacks of cardiac failure, dyspnoea, dropsy, etc., and each time the rest in bed and digitalis may suffice to restore compensation. A fourth or fifth, even a sixth, attack may be safely weathered, and then the final breakdown occurs when nothing avails to combat the dilatation. Of substitutes for digitalis, caffeine and convallaria have been much used of late. Caffeine in some cases acts more promptly, which is an advantage, but its action is not so certain and not so enduring. Convallaria is very variable {636} in its action; it has succeeded in some instances in which digitalis has failed, and in others has been quite without effect. In extreme cardiac failure with great dilatation, lividity, orthopnoea, and feeble pulse, stimulants must be freely given; ether may be employed hypodermically. In this condition of final asystolism digitalis seems to have lost its influence. In the heart failure of pneumonia I have found camphor a valuable adjuvant to the diffusible stimulants. To improve the general nutrition, and with it that of the heart-muscle, iron and arsenic are most valuable adjuvants, especially in the dilatation of anæmia. The treatment of special symptoms, dropsy, dyspnoea, etc., is considered under Valvular Affections.

[Footnote 69: In Ortel's system (Ziemssen's Handbuch der Allgemeine Therapie, Bd. iv.) of treating heart disease exercise, particularly climbing, forms a very important part, but an analysis of his cases shows that most of them were instances of fatty heart in obese persons. It would scarcely be applicable to valvular disease. The severe exercise, he thinks, stimulates the heart-muscle and helps in the restoration of the hypertrophy. His other suggestion, the reduction of the liquids ingested, seems much more reasonable, as in this way the volume of blood to be circulated may be considerably reduced.]

Aneurism of the Heart.

This term is now restricted to local or partial dilatations of the wall of one of the cardiac cavities. Formerly, dilatation of the heart or of one of its chambers was spoken of as aneurism. This rare condition[70] is most frequently associated with fibroid degeneration, but other causes of local weakness of the walls, as ulcer, acute myocarditis, and fatty degeneration, have been present in a few cases. An instance is on record where the aneurism followed a stabbing wound of the chest.[71] The left ventricle is usually involved; very few cases occur in the other chambers. The condition may be acute or chronic.

[Footnote 70: In the index catalogue there are references to only 18 cases by American authors. In the museums of Philadelphia there are only 5 specimens--3 in the museum of the College of Physicians; 1 each in the University and Pennsylvania Hospital cabinets.]

[Footnote 71: Quoted by Legg, Bradshawe Lecture on Cardiac Aneurisms, London, 1883.]

Acute aneurism is met with occasionally in ulcerative endocarditis, more rarely as the result of local softening due to myocarditis or plugging of a branch of a coronary artery. In severe endocarditis perforation is, I think, more common than the production of aneurism. In one case I saw a deep excavation at the upper part of the septum produce a bulging the size of a marble in the wall of the left auricle, and in another ulceration in one sinus of Valsalva had extended into the septum, the upper part of which presented an aneurismal dilatation which had ruptured into the left ventricle. Legg considers the production of acute aneurism by the rupture of abscesses or cysts as doubtful.

Chronic aneurism is almost confined to the left ventricle, and, as Cruveilhier pointed out,[72] is the result of fibroid degeneration of the muscle. In a few instances fatty degeneration appears to have been the cause. The monographs of Thurnam,[73] Pelvet,[74] and Legg[75] give the most complete account of the disease. They are more common in men than in women, and the majority of the cases occur after middle life.

[Footnote 72: Anatomie pathologique, Paris, 1835-42.]

[Footnote 73: Medico-Chirurgical Transactions, vol. xxi., 1838.]

[Footnote 74: Des Aneurysmes du Coeur, Paris, 1867.]

[Footnote 75: Loc. cit.]

The situation of the aneurism is most frequently at the apex--59 of 90 cases collected by Legg. They are usually rounded in shape, and may vary in size from a marble to a cocoanut. The sac may be double, as in a case described by Janeway,[76] or, as in a specimen in Guy's Hospital Museum, the whole wall of the ventricle may be covered with aneurismal bulgings. In the simplest form there is a rounded dilatation at the apex, and the lower part of the septum is lined with thrombi. Often the tumor is distinctly sacculated, and communicates with the ventricle by a very small orifice. The pericardium is usually thickened, and calcification may occur in the walls. Rupture seems rarely to occur--in only 7 of the 90 cases collected by Legg. Of other {637} parts of the ventricle, the septum and the undefended space at the highest part of the septum just below the aortic ring are most often involved. This latter situation is sometimes the seat of a congenital dilatation, usually a small, thin, smooth sac without thrombi, which has no pathological significance.

[Footnote 76: N.Y. Med. Journ., 1875, xxi.]

Cardiac aneurisms rarely produce any symptoms, and in the majority of cases have been found accompanying other conditions which have proved fatal. At the left apex the increase in dulness and area of pulsation could scarcely be distinguished from hypertrophy unless associated with marked bulging. They seldom perforate the chest-wall. Berthold (quoted by Legg) has described one connected with the right auricle which produced a pulsating tumor beneath the skin, the region of the second and third ribs.

Adventitious Products in the Heart.

Tubercle.--In general tuberculosis and in tuberculous pericarditis there may be nodules in the heart-substance, but, as a rule, this organ is very rarely the seat of tubercle. Large caseous masses sometimes occur, but unless associated with tubercle in other organs they are not to be regarded as necessarily tuberculous. Miliary granulations have been seen on the valves.

Cancer and sarcoma rarely are primary, and are not often met with as secondary growths. Sometimes a mediastinal sarcoma penetrates along the veins and involves the auricle, with or without great involvement of the pericardium. The secondary tumors may be single or multiple. In a case of cancer of the uterus I found a large mass in the wall of the right ventricle, involving also the anterior segment of the tricuspid, and partially blocking the orifice. The surface was eroded, and the pulmonary arteries contained numerous cancerous emboli. In another instance the heart was considerably enlarged by the presence of many rounded masses of colloid cancer throughout the walls. In a remarkable case of sudden death in a child I found the tricuspid orifice firmly blocked with a sarcomatous mass which I thought at first had originated in the heart, but dissection showed to have come from the renal vein, which was filled with sarcoma extending from a large tumor of the kidney. Melanotic cancer, fibromata, and myomata have occasionally been seen, and a secondary epithelial growth has been described by Paget.

Syphilis of the heart is met with in the form of gummata or as a specific arteritis leading to patches of fibroid induration. The gummous growths form tumors of variable size, which usually occupy the septum or the ventricles. Possibly many of the caseous and calcified masses not infrequently met represent obsolete gummata. The syphilitic myocarditis probably originates in an affection of the arteries, and leads to patches of fibroid induration more or less extensive. Many authors hold that syphilis plays a very important rôle in the production of fibroid heart.

Cysts.--Simple cysts are rare in the heart. I have met with two instances--one, the size of a marble, situated in the wall of the right auricle near the septum, was filled with a brownish fluid; the other, the size of a small walnut, occupied the base of the posterior segment of the mitral, and was filled with a clear fluid. Blood-cysts occasionally occur.

Parasites.--The Cysticercus cellulosæ, the larva of Tænia solium, and the hydatid or echinococcus, the larva of Tænia echinococcus of the dog, are sometimes found in the heart. The former, usually single, is extremely rare; in the hog and calf the measles, as the cysts are called, very often exist in the heart-muscle. In the recent paper by Mosler[77] references are given to 13 cases of cysticerci in the heart. The greatest number present was 19. The {638} hydatid is more common: 25 instances are mentioned in the statistics of Devaine and Cobbold, and Mosler's more recent figures only give 29. They occur in the right ventricle more frequently than in the left. Occasionally they attain a larger size and compress the heart and push back the lungs. The cyst may burst and the contents be discharged into the pulmonary artery or aorta, as in a case given by Osterlen,[78] in which gangrene of the right leg followed the plugging of the femoral by hydatid vesicles discharged into the blood by the bursting of a cyst in the left auricle.

[Footnote 77: Zeitschrift für klinische Medicin, Berlin, Bd. vi., 1883.]

[Footnote 78: Virchow's Archiv, xlii.]

{639}

ENDOCARDITIS AND CARDIAC VALVULAR DISEASES.

BY ALFRED L. LOOMIS, M.D.

Endocarditis.

DEFINITION.--Endocarditis is an inflammation of the endocardium, and may be either exudative, neoplastic, or ulcerative in character. While its different varieties are closely connected in their etiology, they are distinct in the extent, duration, character, and course of their pathological changes. They cannot be classified as acute and chronic in the ordinary acceptation of these terms, for they often so merge into each other as to render it difficult, if not impossible, to determine when they cease to be acute and become chronic; and some cases are at no time acute. It has been claimed that an acute endocarditis becomes chronic when its course is prolonged, but the advanced changes are only a stage of the acute process.

So-called acute endocarditis is accompanied by a fibro-cellular exudation into the substance of, and underneath, the endocardium, causing elevations of its surface. The better term for this variety is exudative endocarditis, it being borne in mind that the exudation does not take place upon the free surface of the membrane, but into its substance and underneath it. This form of endocarditis may be entirely recovered from, or it may lead to interstitial changes in the endocardial and myocardial tissue which will correspond to the changes usually described as those of chronic endocarditis.

Interstitial endocarditis is a better term for these changes. The disease may be the sequela of exudative endocarditis, or may be interstitial from its commencement, for the valvular changes of interstitial endocarditis are often found in those who never have had either acute articular rheumatism or exudative endocarditis, but have been the subjects of chronic rheumatism or gout.

Acute exudative endocarditis may, in certain cases, be stamped with an ulcerative process, the result of septic infection, giving rise to those pathological changes which have been described as acute ulcerative endocarditis.

HISTORY.--The history of endocarditis is restricted to modern pathology. It is not spoken of by the older medical writers. Before the sixteenth century knowledge of the structure and functions of the heart was imperfect and scanty, and its diseased conditions were altogether unknown.

The history of the pathology of cardiac disease commenced with Harvey, Lancisi, Vesalius, and Vieussens. They investigated not only the normal structure of the heart and the mechanism of the circulation, but accurately described a few of its valvular diseases.

There is little doubt but that Laennec, Senac, and Morgagni were quite familiar with the valvular diseases of the heart, but Kreisig first traced the relationship between valvular diseases and inflammation of the lining membrane of the heart.

{640} The term endocarditis was first used by Bouillaud, who had the advantage of Laennec's discovery of auscultation. Corrigan first discovered the physical signs of aortic insufficiency. The most important advance in the pathology of endocarditis is due to the investigations of Virchow and Luschka, the former developing its sequelæ or results, the latter its histological changes. Ulcerative endocarditis is of modern date, and its literature scarcely extends back twenty years. The labors of Kirk, Virchow, Charcot et Vulpian, Moxon, Eberth, and Lancereaux are all connected with the etiology and anatomical changes of ulcerative endocarditis.

The relationship of interstitial endocarditis to valvular diseases of the heart and to cardiac murmurs is a subject which at present is engaging the attention of many medical observers.

I shall describe endocarditis under three heads: 1st, Exudative endocarditis; 2d, Ulcerative endocarditis; 3d, Interstitial endocarditis.

That the pathological changes which I shall describe may be readily appreciated, I will briefly review the anatomical structure of the endocardium.

The endocardium consists of connective tissue, with numerous elastic fibrils, covered by and continuous with a layer of flattened cells. Upon this lies the endothelial layer, which disappears in twenty-four hours after death.

Luschka regards the endocardium as continuous with all the arterial tissues, but the majority of histologists consider it a continuation of the internal membrane. Some regard the endocardium and inner coat of the arteries as analogous, since both are non-vascular and have an endothelial covering upon a connective-tissue base. As endocarditis is, for the most part, limited to the valves of the heart, a knowledge of their anatomical arrangement is important.

A transverse section of a segment of an auriculo-ventricular valve shows that upon the superior or auricular surface and upon the inferior or ventricular surface there are flattened cells and endothelium, and that next to each lies a fibro-elastic layer, the superior being the thicker. These two layers are separated by connective tissue.

The layer of flat cells is thickest on the ventricular surface. The fibro-elastic tissue is thickest at the base of the valve. The semi-lunar valves have endocardium on one side and the tunica intima on the other.

Although the endocardium has no vessels of its own, the capillaries upon the cardiac walls are in contact with it. The arrangement in the valves is different, as only a few vessels ramify between the layers of the mitral valve, and none are found, normally, in the sigmoid valves.

Acute Exudative Endocarditis.

This variety of endocarditis is met with most frequently in connection with acute articular rheumatism.

In adults it usually has its seat in the left heart; in intra-uterine life it occurs in the right heart. The inflammation commences in, and seldom extends beyond, the valves and the valvular orifices, but it may involve the whole or any part of the ventricular or auricular portions of the endocardium.

MORBID ANATOMY.--The endocardium becomes infiltrated with young cells, the process beginning in the layer of flat cells. The new formative cells are developed not only from the cells of the layer immediately underneath the endocardium, but also from leucocytes. This hyperplasia, this heaping up of embryo-plastic cells, is accompanied by softening of the deeper {641} layers of the intercellular structure, and as the softening goes on the intercellular substance is destroyed.

The endothelial elements also play an active part in the processes. The masses of new cells push out the endocardium, and papillary elevations are formed, filled with a fluid whose chemical properties resemble those of mucin, since it coagulates into threads when acetic acid is added. The cone-like vegetation is surrounded in the deeper layers of the endocardium by a zone of proliferation which is never distinctly limited, but which exhibits progressive hyperplasia from the periphery toward the centre.

All these changes may have taken place in non-vascular tissue. Where the capillaries are most numerous a punctuate or arborescent vascularity is seen, and this is followed by opacity of the part which is the seat of the inflammation. After death the endocardium and lining membrane of the vessels are often stained; this staining is produced by the coloring matter from the red corpuscles, and is the result of post-mortem change.

There is no exudation upon the villous projections; the coagula found upon them are a deposit of fibrin from the blood, the projections acting as foreign bodies in the blood-current. The fibrinous deposits occur chiefly on the surface which is opposed to the current of the circulation, and sometimes they are distinctly conical; at others they have the shape of a raspberry. They occupy the parts most exposed to the friction of the blood, and are arranged on the borders of the aortic valves at a little distance from their edges, the seat being determined by the limit of the vascular network. The band of tissue which passes from the attached border of the valve to the Arantian body in the centre shows the inflammatory granulations most distinctly. They consist of a cauliflower-like bulbous extremity, connected by a constricted neck with a firm, hard base that is intimately blended with the subjacent tissue. A thin hyaline layer covers each mass. At first these granulations or vegetations are very small and numerous, so that the membrane presents a granular appearance. Later, they become larger, reaching oftentimes the size of a small pea.

Near the insertion of the tendons upon the auricular surface of the mitral valve are found irregular wreaths of vegetations which enclose the attachments of the chordæ tendineæ. Moxon has shown that the friction of the vegetations or of fibrinous clots that gather upon the vegetations may, by the irritation it produces, excite endocarditis at points remote from the valves.

The tendon of the mitral valve may show the effects of endocarditis by becoming soft and friable, and even rupturing, or the chordæ tendineæ may adhere to one another. When such adhesions occur either with agglutinations of the flaps to each other or to the heart-walls, stenosis or regurgitation may result.

In connection with these changes new vessels are developed in the substance of the mitral valve, or those that already exist become more apparent. In the semilunar valves new vessels are formed or neighboring capillaries send out prolongations into the parts destitute of vessels. This, according to Charcot, is one way in which arborescent vascularity occurs. These changes are most marked in those forms of exudative endocarditis which run an acute course.

In some instances the hyperplasia is so extensive as to interfere with nutrition, and may lead to fatty metamorphosis. A cavity is then formed filled with granular fat-cells, discrete fat-globules, and blood-pigment, whose endocardial covering ruptures, and the contents are carried into remote capillaries to cause capillary embolism and septicæmia. This has been called ulcerative endocarditis.

{642} Ulcerative Endocarditis.

Ulcerative endocarditis occurs in those diseases where there is great vital depression. It is met with oftenest in pyæmia, puerperal fever, scarlatina, and diphtheria. It has been called septic, diphtheritic, and infectious endocarditis.[1]

[Footnote 1: Jaccoud, Klebs.]

MORBID ANATOMY.--Ulcers may form in endocarditis in either one of three ways: 1st. The exudative process may be so rapid and extensive as to cut off the nutrition of the endocardium covering the apices of the papillary elevations, and ulcers result in non-septic inflammation. 2d. Degeneration of the neoplastic tissue, due either to deficient blood-supply or other causes of impaired nutrition, may so soften the villi or efflorescences that their apices will be swept away by the blood-current and ulcers thus be formed. Charcot especially insists that the ulceration of these elevations is the consequence of granular degeneration, and not of fatty metamorphosis, with which it is often confounded. 3d. The exudative process may be purulent in character, and form minute abscesses in the substance of the valves beneath the endocardium, which, rupturing, leave comparatively deep ulcers. Acute multiple abscesses in the aortic valves are of frequent occurrence in ulcerative endocarditis.

The margins of the ulcers are irregular, but well defined; the edges are swollen and thick, and their floor (the muscular substance of the heart or the fibrous layer of the valve) is infiltrated with pus.

Where there is extensive loss of substance perforation of the valve may occur. These perforations are sometimes closed or hidden by a fibrinous exudation.

The soft and friable vegetations may be torn into long shreds by a forcible blood-current, and subsequently may excite endocardial inflammation where they come in contact with the walls of the heart-cavity, or they may break off and form emboli. A fibrinous string upon a flap of the aortic valve is not infrequently driven down and back by a regurgitant current, so as to excite endocarditis in the mitral valve.

Some observers state that micrococci and bacteria are found in ulcerative endocarditis of a septic or diphtheritic origin, and they have given to it the name of mycosis endocardii. It is probable that these minute organisms are developed by the septic ulcerative process rather than that they are the cause of such processes. They appear as spheres, highly refractive, motionless, cohering in groups, without any stroma. Acids, alkalies, ether, and chloroform have no effect on them, so that they are not to be regarded as vegetable products.

The valvular ulcerations in this form of endocarditis give rise to the most diverse lesions. Masses may be detached from the diseased cardiac orifices, either from the fibrinous deposits on the valves or from ulcerations of the valves themselves, and, having entered the circulation, they will produce various symptoms in the organs and tissues to which they are carried.

It is important to make a distinction between the results produced by displacements into the blood-current of large masses and those arising from the entrance of molecular fragments. It is also to be remembered that the masses from the vegetations or ulcerated valves in ulcerative endocarditis are often stamped with a septic element which leads to the development of suppurative infarctions in different organs.

The size and site of the emboli are important, for they may be so large as to obstruct vessels of large size.

The femoral and even the external iliac may suddenly become impervious to the circulatory currents, on account of the presence of a large embolus from the heart.

{643} When the arteries in the limbs are thus plugged, the result is generally an ischæmia, terminating often in gangrene. Capillary embolism may occur in a number of organs at the same instant, and give rise to a variety of lesions. When the cutaneous capillaries are obstructed ecchymotic spots are produced, followed by cellulitis. When the cerebral vessels are obstructed softening may occur, which, if the vessels are very small, may be developed without any evidence of obstruction to the cerebral circulation. If the obstructed artery is of large size, instantaneous hemiplegia and secondary softening will result.

Capillary emboli may have their seat in the vessels of the spleen, giving rise to infarctions and suppuration.

The kidneys may also undergo analogous changes. Rayer, without knowing the origin of these changes, has given an excellent description of them under the name of rheumatic nephritis.

In addition to the local lesions arising from these arterial or capillary emboli, the septic phenomena are most important. When typhoid symptoms, deep jaundice, and symptomatic intermittent fever are associated with acute endocarditis, it establishes its ulcerative character. In acute exudative as well as in ulcerative endocarditis, when the inflammation progresses rapidly, the valves soften and become less resistant than normal. As a result, they are stretched, bulged, or torn by the stream of the circulating blood-current.

A rupture of the mitral valves will open into the auricular, and that of the aortic into the ventricular, cavity. The reason for this is to be found in the fact that when the valves are closed the blood-pressure is exerted from the left ventricle toward the mitral valve, and from the aorta toward the semilunar valves. If the blood penetrates a rent in a flap of the valves, the endocardium is puffed out, and a valvular aneurism is formed, and round or funnel-shaped aneurismal sacs may project from the valves. The bottom of one of these sacs may be perforated, and long, ragged, gray shreds, covered with fibrin, may be found hanging in the ventricular cavity.

Microscopically, the torn shreds from a valvular aneurism, the result of acute endocarditis, consist of nuclei and round cells imbedded in a mass of granular matter. There is neither connective fibrilla nor elastic tissue. When the ulceration is localized in the ventricle, the pressure of the blood may bulge out the heart-wall, and thus give rise to a so-called partial cardiac aneurism. By rupture of such aneurism communication between the different heart-cavities may be established, which will vary with the seat of the ulceration.

Acute exudative endocarditis may involve the muscular structure of the heart. Such myocarditis (or carditis) may involve the deeper structures, weaken them, and so alter their consistence that bulging and the formation of a ventricular aneurism may result. Usually such myocarditis is so slight that incomplete organization of the new embryo-plastic cells occurs and the tissue undergoes fatty changes. The results of all forms of acute endocarditis are best studied in connection with the morbid changes of interstitial endocarditis, into which they so often gradually merge.

Interstitial Endocarditis.

MORBID ANATOMY.--Interstitial (or chronic) endocarditis may be a continuation of a process which commenced in an acute exudative endocarditis, or it may be interstitial from its commencement, and be so insidiously evolved as to escape notice. The anatomical changes may sometimes be confined to the edges of the valves, at others to their base, or they may involve the entire valves, which become thickened, indurated, contracted, degenerated, and {644} adherent. It is more closely allied to rheumatism, gout, and chronic interstitial changes in other organs than either of the other varieties.

There is no part of the endocardium which is exempt from interstitial inflammation. The favorite place for its development is the endocardium of the valves and that at the apex of the left ventricle. The thickening at first may be either translucent or opaque, and the valves may become three or four times thicker than normal. In some instances, although the valves are thickened and indurated, their functional activity is not interfered with, and they offer no obstruction to the blood-current.

White, thickened, opaque spots are often irregularly scattered over the internal wall of the heart. The vegetations met with in interstitial endocarditis differ from those of the acute exudative variety in that they are less prominent and firmer. They rest upon an indurated base. Their cartilaginous consistency is due to the fact that their cellular elements are not round (as in acute exudative endocarditis), but elongated and flattened, possessing an abundant intercellular fibrillated tissue.

In and underneath the endocardium there is an increase of tissue, and upon any prominence arising from the thickening of the endocardium occur fibrin deposits. These fibrinous efflorescences assume a variety of forms, and sometimes string out into the adjacent vessels and cavities for half an inch or more. Their usual form is globular or wart-like, and their seat is on the ventricular surface of the aortic and upon the auricular surface of the mitral and tricuspid valves.

In interstitial endocarditis the cell-development is far less rapid and abundant than in the acute exudative form, and this very slowness accounts for the greater induration and thickening.

A microscopical examination of a cross-section of an indurated valve shows a number of flat cells arranged in irregular layers, having between them a fibrinous material which has in it here and there a few elastic fibres. The new formations always originate in the layer of flat cells. These changes are best marked in the fibrous zone at the valvular orifices, upon the surfaces of the valves themselves, and in the chordæ tendineæ. The new tissue, whether developed rapidly as in acute exudative, or slowly as in interstitial endocarditis, becomes fibroid and contracts, and this contraction is progressive.

As a consequence, the rigid valves, whose edges are round and hard, are drawn toward their base, and thus are made to assume a puckered appearance. A similar process in the chordæ tendineæ causes them to become hypertrophied, rigid, and cartilaginous, while they are diminished in length. In this way the valves are not only diminished in depth, but not infrequently have their free edges approximated to the cardiac walls, so that extensive valvular insufficiency is the result. This, however, does not always happen, for a thickened cartilaginous valve may have such abundant fibrinous or papillary excrescences upon it that the onward current is obstructed and extensive stenosis results.

As the thickening and rigidity of the flaps of a valve increase, their mobility is diminished, and adhesions take place between their edges which begin at their bases and progress toward their apices: so thoroughly do they become adherent that in some cases all evidence of a valvular outline is lost, and a fibrinous diaphragm is formed across the valvular orifice having only a small slit at its centre, looking and feeling like a buttonhole; hence the term buttonhole slit. The mitral opening, which will usually admit the ends of three fingers, may be so narrowed that the end of the little finger will scarcely pass through it, and the aortic opening may become so diminished as not to admit a small quill. These retractions and adhesions cause the mitral valves, with their columns and cords, to assume the form of a perforated cone.

{645} Long stringy masses of fibrin, when located on the aortic valve, sometimes form adhesions with the aortic walls, and thus is induced a sudden and extensive regurgitation.

Insufficiency and stenosis are often found at the same valvular orifice as the result of the thickening, adhesion, and retraction.

Changes at the aortic orifice usually occur after middle life, and induce more insufficiency, retraction, and adhesion than those which are limited to the mitral valve. The mitral valves are the most frequent seat of interstitial endocardial changes in early and adult life. These lesions are analogous to those characteristic of endarteritis deformans. The tendency of the lowly-organized tissue which results from interstitial endocarditis is to undergo fatty and calcareous changes.

The minute patches of fatty degeneration in the imperfectly organized tissue underneath the endocardium sometimes form atheromatous masses containing more or less granular débris. The endocardium over these patches may be destroyed, or the patches may soften and ulcerate and cause extensive destruction of the valves. Valvular aneurism may form in the same manner as has been described in exudative endocarditis. The formation of calcareous granules and plates is a very frequent termination of interstitial endocarditis.

The aortic orifice is the most frequent seat of calcareous degeneration. It is rarely associated with mitral stenosis. So extensive may this process be that little beads of chalky material may be seen studding the free edges of the valve and even extending into the cardiac cavities.

When interstitial endocarditis has its seat in the endocardium of the cardiac cavities, the endocardium will undergo changes similar to those of the valves, and the muscular walls of the heart will be the seat of interstitial myocarditis. As a result, the walls of the heart become thinner and less resistant than normal, and depressions are formed on its inner surface. The process is in reality a fibrous overgrowth, which occurs in spots varying in size from half an inch to one inch in diameter. When it extends through the entire heart-wall the columns and cords may be so shortened as to cause valvular insufficiency.

If the cardiac walls yield so that a well-defined pouch is produced, a condition results which is called aneurism of the heart. Cardiac aneurism, thus induced, is usually seated at the apex of the left ventricle; the aneurismal sac may vary in size from that of a marble to that of a closed fist, and may communicate with the ventricle by a funnel-shaped or ring-like aperture. The walls of the sac are solid and rigid; the internal surface is smooth, but it may be anfractuous. In the latter case clots adhere to its wall. Cardiac muscular fibres are found here and there in the aneurismal walls. They are mostly, however, made up of layers of flat cells, their flatness being the result of pressure.

Aneurisms at the base and in the inter-ventricular septum may result from the extension of a valvular aneurism.

ETIOLOGY.--In most instances endocarditis depends upon a constitutional dyscrasia characterized by alterations in the vital, physical, or chemical properties of the blood.

Acute exudative endocarditis rarely, if ever, occurs as a primary or idiopathic affection. It seems to have a direct connection with those diseases and dyscrasiæ in which the blood is altered either in the relative proportions of its constituents or in its physiological elements. So frequently is acute exudative endocarditis associated with acute articular rheumatism that they have often been described as one disease.

It is generally stated that acute endocarditis occurs in 50 per cent. of those who suffer with acute articular rheumatism, but the statistics of Bellevue {646} Hospital show that endocarditis complicates rheumatism in only 33 per cent. of the cases. From these statistics it is evident that a majority of the cases of acute rheumatism run their course without endocardial complication.

The irritant action of the blood, the salts of which are changed or which contains excrementitious products or a specific poison, is shown most markedly upon the valvular surface of the endocardium; and it is for this reason that the parts which are most exposed to friction of the blood-current are those which first and most extensively exhibit the pathological changes of endocarditis.

Charcot records a large number of observations in which endocarditis developed in patients with chronic rheumatism and in which it never assumed an acute form. It therefore seems evident that organic lesions of the valves from endocarditis may occur in the course of chronic as well as of acute rheumatism.

There is no disease in which a morbid blood-state exists in which endocarditis may not occur. The essential fevers, the exanthemata, diphtheria, septicæmia, pyæmia, and Bright's disease, are all conditions in connection with which endocarditis is frequently exhibited. It is met with occasionally in secondary syphilis.

Acute and chronic Bright's disease are often complicated by it. When an individual who is already the subject of valvular disease of the heart is attacked with acute rheumatism, the liability to endocarditis is much increased.

Even when rheumatism and chorea are absent, endocarditis is liable to occur when valvular disease exists. Some regard myocarditis, pericarditis, pleurisy, and pneumonia as capable of exciting endocarditis by the extension of the inflammatory process from the surface of the heart; it is questionable if it ever results from such extension. That it can be the result of traumatism is possible: Bamberger records two cases of traumatic endocarditis. Wunderlich ranks measles, next to rheumatism, as a cause of endocarditis.

In estimating the etiological importance that any disease bears in the production of endocarditis, we must remember that not every blowing sound or murmur is indicative of an inflamed endocardium. Bamberger and Niemeyer think that the excited and irregular action of the heart in children, by inducing irregular tension of the valves, may bring about a blowing sound during the course of acute rheumatism.

Acute ulcerative endocarditis is met with in pyæmia, puerperal fever, and endometritis, scarlatina, and diphtheria: it may occur as a secondary affection to some inflammatory focus located in the body--septic endocarditis.

Again, this form of endocarditis may appear without obvious cause--spontaneously or in connection with some specific form of inflammatory disease, as croupous pneumonia. Wilks calls it then arterial pyæmia. Primary ulcerative endocarditis is a name recently and perhaps more aptly given it.

Finally, ulcerative endocarditis may appear as a graft (recurrent endocarditis) upon a valve the seat of interstitial endocarditis, and have all the pathological appearances of the septic form, but none of its clinical aspects.

The majority of cases of interstitial endocarditis are the sequelæ of the exudative form. It is far more frequently associated with articular rheumatism than with any other condition. In a certain proportion of cases the process is interstitial from its onset, especially when it occurs with gout, chronic rheumatism, in alcohol-drinkers, or in the aged.

SYMPTOMS.--The subjective symptoms of acute exudative endocarditis are more obscure than those of any other disease. They are not only few and ill-defined, but they have no regular order of development. When the muscular tissue of the heart is not involved the disease may run its entire course without exhibiting a single subjective symptom.

{647} The urgent symptoms of acute rheumatism, the different phases assumed by the dyscrasiæ and acute infectious diseases in which this condition is liable to occur, so mask those of the endocardial inflammation that they are often overlooked.

When the endocardial inflammation is extensive and the muscular tissue of the heart is involved, the patient will complain of palpitation and a sense of discomfort in the region of the heart; not infrequently cardiac palpitation is accompanied by dyspnoea, and decubitus on the left side is noticed. In a small percentage of cases the palpitation is appreciable to the physician. The heart may beat with great force and its action be tumultuous, and yet the pulse not be altered in character.

The pulse, at first, is usually strong and forcible; later, it becomes rapid, small, feeble, and irregular. In some cases it is very frequent from the onset of the disease. As a rule, the force of the pulse will not correspond to the cardiac activity; for, as the muscular fibres of the heart become involved, its propelling power is diminished, and the pulse is correspondingly feeble and compressible. It may be dicrotic. The respirations are more or less accelerated, and sometimes labored, and there may be paroxysmal dyspnoea. The face may be flushed and covered with a profuse perspiration, or it may assume a dusky, pallid, ashy-gray, or slightly cyanotic hue. In rare cases there may be sleeplessness or nocturnal delirium of a typhoid type. If the muscular tissue of the heart is extensively involved, nausea, vomiting, giddiness, and syncope may be present.

When there is pain in the cardiac region, especially if it is augmented by pressure, pericarditis is usually present, and slight pain or tightness in the cardiac region is not an infrequent symptom, and is quite common when endocarditis occurs in those who are the subjects of chronic valvular disease.

The temperature in acute exudative endocarditis seldom exceeds 103° F.

When ulcerative endocarditis complicates septicæmia and a rupture of a valve occurs, a typhoid state rapidly supervenes. The patient is forced to assume the sitting position on account of the intensity of the dyspnoea, cyanosis is sudden and extreme, and the symptoms of multiple embolism make their appearance. The febrile symptoms are marked; the temperature may reach 106-107° F.; the patient becomes jaundiced; and there are frequent rigors, which, with the paroxysmal febrile attacks, simulate the icteric form of malarial fever. The spleen becomes enlarged and tender, the urine becomes scanty, dark-colored, albuminous, and of high specific gravity, and in severe cases delirium and coma occur.

Some cases of endocarditis putrida (as some German pathologists call it) are attended with nausea, vomiting, and diarrhoea. The frequency with which this form of endocarditis is associated with pneumonia certainly suggests a blood-poison of great intensity. Although it is rarely met with except in septic conditions, it may occur late in severe forms of rheumatic and traumatic endocarditis or when there has been pre-existing suppurative disease of the bones.

The symptoms which attend embolism from detachment of the fibrinous efflorescences upon the valves are due to the arrest of such a plug in an artery whose calibre is too small to admit of its passage. Beyond the obstruction the circulation is arrested; hence results either an infarction or necrosis of the part whose blood-supply is thus shut off. The organ most liable to be the seat of such emboli is the spleen, and after this the kidney and the brain. Hence the occurrence of hemiplegia with aphasia or marked cerebral symptoms in the course of acute endocarditis is indicative of cerebral embolism.

There are no positive subjective symptoms of interstitial endocarditis. There may be palpitation and a sense of uneasiness, sometimes amounting to {648} pain at the præcordial region, with irregularity in the action of the heart, but all of these, when taken together, are not sufficient for a diagnosis. This can be made only from changes in the heart-sounds produced by changes in the valves and valvular orifices.

PHYSICAL SIGNS of exudative endocarditis.--Inspection.--Upon inspection it will sometimes be noticed that the area of the cardiac impulse exceeds the normal--that it is irregular and often tumultuous. As the disease advances, the apex-beat and the impulse grow more indistinct, but never to the same extent or so suddenly as in pericarditis. In children the vessels of the neck exhibit venous stasis far more frequently than in adults.

Palpation.--At the onset of an endocarditis the cardiac impulse is more forceful than normal, and the heart-action is frequently irregular. In some instances the heart thumps violently against the chest-walls. The force of the cardiac impulse varies from day to day. The impulse is stronger when pain is present over the præcordial space. If during the entire course of the disease there is no decrease in the force of the apex-beat, it may be inferred that there is no deficiency in the muscular power of the heart. When acute endocarditis supervenes upon long-standing valvular disease, there will be an alternate increase and diminution in the area and force of the impulse. When the walls of the heart become weakened by subsequent myocarditis, or when the endocardial inflammation is itself very extensive, the force of the apex-beat is diminished. An endocardial thrill is frequently present in acute exudative endocarditis.

Percussion.--The area of cardiac dulness in endocarditis is normal, unless changes at the valvular orifice retard the outflow of blood from the lungs, and then the right-heart cavities become engorged and the area of dulness will extend beyond the normal limits. But it is to be remembered that the increase is always slight, except in those few cases where the heart-cavities are both suddenly and extensively distended with blood or masses of fibrin. Extensive myo- or endocardial inflammation may so weaken the heart-walls that they will dilate, and then percussion will reveal an enlargement in the area of cardiac dulness.

Auscultation.--On auscultation a murmur or murmurs can be heard over the various cardiac orifices. The fact that valvular disease may have previously existed makes it important, at the first visit to a patient who is suffering from acute articular rheumatism, chorea, Bright's disease, etc., to carefully examine the heart. When cardiac hypertrophy exists and valvular disease has pre-existed, it is difficult, if not impossible, to recognize acute exudative endocarditis or to determine the time of its advent if it exist. The most important and constant sign of endocarditis is a systolic murmur, its greatest intensity being over the apex; but this murmur, which is soft and blowing in character, the so-called bellows murmur, may be either ventricular or valvular. In all cases it is due to roughening or thickening of the endocardium. It often changes its point of maximum intensity during the acute period of the disease. It is developed at the onset of the disease, and when one is on the lookout for endocarditis, this will be the first evidence of its occurrence. And yet in some instances no murmur may be present during the entire course of an endocarditis.

A mitral murmur alone occurs in about 50 per cent. of cases of rheumatic endocarditis. It is usually developed early, and before it becomes distinct it is preceded by prolongation of the first sound. This is a transition sound between a normal heart-sound and a murmur. It is a feeble, wavering sound, extending over the slight interval which normally exists between the first and second sounds.

Other changes that are not murmurs, but which frequently precede them, are loud, ringing normal sounds, muffled first sound, feeble first and {649} intensified second sound, doubling of the first sound, roughness of the first sound, and a humming over the right heart.

Complete absence of the heart-sounds is a rare but possible antecedent of an endocardial murmur. A mitral murmur in acute endocarditis is usually audible over a limited area. It is the exception to hear it both in front and at the back. Very frequently it is heard most distinctly over the stomach.

When the blood becomes dammed back into the lungs, there is an extra strain upon the pulmonary semi-lunar valves, and then the second sound will be accentuated over these valves on account of the sharp shock which they sustain during diastole. With this accentuation of the second sound over the pulmonary orifice, the first pulmonic sound may be feeble or absent. A subdued or absent first sound shows tension of the artery.

Reduplication of the second sound in a mitral endocarditis is probably due to the difference in time occupied by the ventricles in emptying themselves. A tricuspid murmur occurs in 50 per cent. of the cases of acute mitral endocarditis--a pulmonic in about one-third of the cases. They are superficial and scratchy in character, and indicate a relaxed condition of the vessels and a thin condition of the blood. These murmurs are never permanent. Mitral endocarditis is accompanied by aortic murmurs in about 16 per cent. of cases. Acute mitral endocarditis occurring with chorea is as apt to become interstitial as when it is of rheumatic origin.

Aortic murmurs are usually soft and blowing, but they may be musical, whistling, or twangy. In aortic endocarditis the second sound is usually lost over the carotids. Incompetency of the aortic valves is met with only in the interstitial form of endocarditis.

In about 12 per cent. of the cases of exudative endocarditis arising from rheumatism a regurgitant murmur will be heard at the tricuspid orifice, but such murmurs are not the result of endocarditis of the right heart.

Tricuspid murmurs are present in 50 per cent. of all cases of recent mitral murmurs, in about 40 per cent. of recent aortic murmurs, and in about one-fourth of mitro-aortic murmurs. Such tricuspid murmurs are due to an increase in the slight normal insufficiency existing at the tricuspid orifice. They are of short duration, and are heard over the body of the heart over the right ventricle. Sometimes they are vibrating in character.

In children aortic endocarditis is rare; at this period obstruction at and regurgitation through the mitral orifice commonly occur together.

The physical signs of interstitial endocarditis are such as are due to those changes in the valves which will be considered under the head of Cardiac Murmurs, and their Relations to Valvular Diseases.

DIFFERENTIAL DIAGNOSIS.--Acute exudative endocarditis may be mistaken for pericarditis, and its murmur may be mistaken for the murmur produced by aortitis and for those that develop during the course of fevers.

The friction sounds of pericarditis are superficial in character, and are limited to the præcordial space, while the murmurs of endocarditis are distant, and each murmur will have its area of diffusion beyond the præcordial space. A pericardial sound is distinctly a friction, creaking, or rubbing sound; it has a to-and-fro character, while the murmur of endocarditis is soft and blowing.

Endocardial murmurs accompany the heart-sounds, while pericardial friction sounds are not always rhythmical with the heart-sounds.

The intensity of a pericardial friction is increased when the patient bends forward at the end of a full inspiration or when the stethoscope is pressed firmly over the præcordial region; and in the last-named case it becomes distinctly grazing and rubbing in character. In endocarditis these methods produce no difference in either the intensity or the character of the murmur. There is an endocardial thrill in endocarditis not present in pericarditis.

{650} As soon as effusion occurs in pericarditis the absence of pain, the alteration in the character of the pulse, the great increase in dulness, and the disappearance of the adventitious sounds will decide the diagnosis.

Aortitis has most of the symptoms of endocarditis, but in addition the pulse is more rapid, the respirations are more hurried, and pain which shoots down the spine and is increased by motion is present in the præcordial region. Not infrequently aortitis is accompanied by cutaneous hyperæsthesia.

Acute inflammation of the aorta is exceedingly rare, and in the few cases observed has been complicated by very grave diseases. Indeed, Powell, Lebert, and Rindfleisch doubt its existence.

In the Medico-Chirurgical Transactions (vol. xlvii. p. 129) Moore gives a case where rigors, fever, intense and painful throbbing of the aorta, and embolic infarction of distant organs occurred, with symptoms so resembling those of endocarditis that few would venture to favor a diagnosis of aortitis during life.

The functional cardiac murmurs which occur in fevers are usually heard only at the base of the heart, while those of endocarditis are most frequent and distinct at the apex. There are no symptoms of obstruction present with febrile murmurs, while they are frequently present in endocarditis.

It is often difficult to determine whether an endocardial murmur is of old or recent origin: if during an attack of acute rheumatism an endocardial murmur is developed under daily examination, it is a certain index of acute exudative endocarditis. If a murmur exists at the first examination which is systolic, soft, and blowing in character, and not accompanied by the evidences of cardiac hypertrophy, there is good reason to believe that it is produced by an acute endocardial inflammation.

If, on the other hand, the murmur is rough in quality, diastolic, and cardiac hypertrophy exists, it cannot be regarded as a sign of acute endocarditis.

The rules for distinguishing murmurs due to interstitial endocarditis from functional murmurs will be given under the head of Cardiac Murmurs.

PROGNOSIS.--Exudative endocarditis is rarely a direct cause of death, but it seldom results in complete recovery. Acute mitral endocarditis terminates in permanent valvular disease in over 25 per cent. of the cases. The elements that will render the prognosis immediately unfavorable in any case are the symptoms of embolism or of metastasis. Sudden splenic enlargement, with tenderness over its site, albuminuria or hemiplegia, when accompanied by the physical signs of acute insufficiency or perforation of a valve with cyanosis, dyspnoea, and disturbance of the heart-rhythm, will render the prognosis exceedingly unfavorable. All these symptoms are diagnostic of acute ulcerative endocarditis, and therefore when the signs of endocarditis appear during the course of pyæmia, diphtheria, or other septic condition, the liability to these conditions must be considered.

When even exudative endocarditis is accompanied or followed by typhoid symptoms its prognosis is unfavorable. In children bronchial complications, catarrhal pneumonia, and intercurrent diarrhoea may lead to a fatal issue. Death may result from acute insufficiency of the heart or from complications.

The prognosis in interstitial endocarditis will depend upon the seat and extent of the valvular lesions which it produces. It will be more fully considered under the head of Valvular Diseases.

In cardiac aneurism death may result from rupture of the aneurismal sac, from apoplexy, or from secondary diseases in other organs.

TREATMENT.--Acute exudative endocarditis is rarely, if ever, idiopathic. It is so constantly associated with certain infectious diseases, and especially with acute articular rheumatism, that its treatment must be determined by the condition under which it occurs.

{651} In every case the patient must have absolute rest in bed in a room whose temperature should never be below 70° or 75° F. The præcordial region should be covered with flannel, and care exercised not to expose the surface when physical examination of the heart is made. Some authorities claim that an ice-bag applied to the præcordial space during the active period of an acute endocarditis will arrest and limit the inflammatory process. My own experience does not sustain the results claimed for this plan of treatment.

In rheumatic endocarditis antirheumatic remedies are indicated, the joints must be kept absolutely at rest, and such local treatment should be resorted to as will relieve pain and give the greatest comfort to the patient.

If the blood is kept alkaline, as indicated by the urine, the liability to endocarditis is diminished.

To ensure rest small doses of opium are often required; but opium cannot be administered as freely in endocarditis as in pericarditis.

During the whole course of acute endocarditis the strength of the patient must be maintained by the judicious use of concentrated nutriment, with some preparation of iron.

When endocarditis occurs with septic diseases and is attended by typhoid symptoms, or when it assumes the ulcerative form, alcoholic stimulants, quinine, and iron must be freely administered.

In endocarditis complicating Bright's disease the rapid elimination of the urea must be established. The severe pain over the præcordial space may be relieved in many subjects by the application of a few leeches to the region.

Experience proved that the employment of mercury (internally) and blue ointment (externally) to lessen the plasticity of the blood, and the internal use of iodide of potassium (for the absorption of fibrinous exudation), were harmful, before it was demonstrated that the theory on which their use was based had no foundation.

Cardiac Murmurs, and their Relations to Valvular Diseases of the Heart.

DEFINITION.--A cardiac murmur is an adventitious or abnormal sound produced within the heart or blood-vessels, either by obstruction to the blood-current, an abnormal direction of the blood-current, or by a change in the blood-constituents.

HISTORY.--The systematic study of cardiac murmurs and valvular diseases dates from the discovery of auscultation by Laennec. Previous to his discovery there are a few recorded cases where observers during the seventeenth and eighteenth centuries described forms of valvular diseases. One of the first to describe a valvular lesion of the heart was Vieussens in 1716. At the close of the seventeenth century Willis and Riverius published cases of valvular disease. In all these instances it was the aortic valves that were diseased, and the discovery of their condition was undoubtedly due to the peculiarity of the radial pulse which is so marked and striking in aortic disease.

In Friedreich's article in Virchow's Handbuch, "Krankheiten des Herzens," Meckel's essay of 1756 is given as the first paper on endocardial disease.

John Hunter[2] in 1794 gives a lengthened account of a most interesting case of aortic valvular disease. Senac[3] gives an account of disease of the auriculo-ventricular valves; and Allan Burns, whose work was published in 1809, describes aortic regurgitation and obstruction, and supposes that "a reflux current can produce a hissing noise, something like what is described as audible palpitation in some diseases of the heart."[4]

[Footnote 2: Treatise on the Blood, etc.]

[Footnote 3: Treatise on the Heart, 1783.]

[Footnote 4: Obs. on some of the most Frequent and Important Dis. of the Heart, Allan Burns, Edinburgh, 1809.]

{652} The subject of vegetations upon the valves was very fully considered by Corvisart in 1806. Corvisart was the first to mention the importance of what is now called the purring thrill. He stated that "it probably came from a difficulty experienced by the blood in going through an orifice disproportionate to the amount of fluid." Laennec regarded murmurs or bruits as the result of spasmodic contraction of the heart or arteries. Corrigan in 1829 defined murmurs as "the result of the development of currents and the intrinsic collision of the moving liquid."

In 1842, Gendrin gave cardiac murmurs as bruits de frottement endocardiaques, and established the friction theory. He also called attention to the fact that alteration in the constituents of the blood will produce murmurs which are heard in arteries of medium calibre.

Bouillaud describes a murmur as an exaggeration of the normal bruit caused by blood-friction against the segments of the heart, and he says that according to the size or condition of the orifice the murmur will be rasping, sawing, or blowing.

Chauveau states that bruit de souffle is produced by the vibration of a nei e fluide, always formed when blood rushes through a part of the circulatory system actually or relatively dilated.

This nei e fluide has its best development in anæmia, when it is termed the bruit du diable, for the jugular veins do not collapse and the volume of blood in anæmia is diminished. Chauveau's theory is applicable to anæmic murmurs, but not to all cardiac murmurs.

Hope states that "valve murmurs are produced by collision of the blood-particles against one another, or that either the liquid alone or the liquids and solids conjointly may develop murmurs."

There are many who have advocated the tension theory--viz. that an increase in tension and force can so exaggerate a normal sound as to produce a murmur. This theory has no clinical foundation. Often, however, valve-lesions may exist, and the blood-current be so weak, the propulsive force so feeble, that no murmurs are audible.

Some observers are of the opinion that spasm of the papillary muscles and chordæ tendineæ and weakening of these structures through fatty degeneration can cause temporary murmurs.

The conditions that determine the character of a cardiac murmur, its pitch, quality, and intensity, are subject to the same physical laws as govern the formation and quality of sound elsewhere. They are the rapidity and force of the moving body, the obstructions which it meets, and the physical properties of the media of conveyance. The same vibration that produces a murmur may produce an endocardial thrill, called sometimes purring thrill.

Far more important, however, than loudness, pitch, or quality of a murmur are its rhythm, its point of maximum intensity, and the area of its diffusion, all of which can best be considered in connection with the physical signs of each valvular lesion.

During a cardiac diastole the heart-cavities are all filling; just before the commencement of the cardiac systole the blood is forced from the lungs and the cavæ through the auricles into the ventricles, while the mitral and tricuspid valves are pressed against the walls of the ventricles, and no obstruction is offered to the blood-current. If, as the result of disease, any obstruction exists at either one of the auriculo-ventricular orifices, the blood as it passes through the opening will impinge on such obstruction and cause a presystolic murmur.

During a cardiac systole the filled ventricles contract; blood is thrown through the semi-lunar openings, the flaps of whose valves are pressed against the walls of the vessels, so that no obstruction is offered to the outgoing {653} current. At the same instant the auriculo-ventricular valves close their orifices, so that blood may not be forced back into the auricles.

If, as a result of disease, the semi-lunar valves should obstruct the outgoing current, or the mitral or tricuspid valves should not wholly close the auriculo-ventricular orifices, then in the one case the direct blood-current, as it passes over the obstruction at the semi-lunar orifices, would produce a systolic murmur, and in the other the backward current through the abnormal opening at the auriculo-ventricular orifice would also produce a systolic murmur.

Again, if the lungs and the aortic system (when filled at the systole) have, back of them, a semi-lunar valve that does not completely close that end of the circuit, the blood will regurgitate into the ventricles during the period of cardiac rest, so that semi-lunar incompetence will cause a diastolic murmur.

ENDOCARDIAL MURMURS.

Rhythm. Situation. Orifice. Nature. Systolic 1 Basic, Aortic, Obstructive. " 2 " Pulmonary, " " 3 Apical, Mitral, Regurgitant. " 4 " Tricuspid, " Diastolic 1 Basic, Aortic, " Presystolic 1 Apical, Mitral, Obstructive.[5]

[Footnote 5: Pulmonary regurgitant murmur (diastolic) and tricuspid obstructive murmur (presystolic) are so rarely met with that, clinically, they may be disregarded.]

The following is the order of relative frequency of cardiac murmurs: 1. Mitral regurgitation; 2. Aortic obstruction; 3. Aortic regurgitation; 4. Mitral obstruction; 5. Tricuspid regurgitation; 6. Tricuspid obstruction; 7. Pulmonary obstruction; 8. Pulmonary regurgitation. The most frequent combinations of murmurs are--1. Aortic obstruction and regurgitation; 2. Mitral obstruction and regurgitation; 3. Mitral obstruction and tricuspid regurgitation; 4. Aortic obstruction and mitral regurgitation; 5. Double valvular disease at aortic and mitral orifices (four murmurs).

It is often difficult, after having satisfied ourselves of its existence, to determine the rhythm of an endocardial murmur. To resolve this difficulty it is necessary to determine which is the first and which the second sound of the heart.

The first sound of the heart is synchronous with the carotid pulse, the radial pulse, and the apex-beat. It may be wholly replaced by a systolic murmur, but the second sound is always heard following the apex-beat, for the pulmonic and the aortic valves are never diseased at the same time.

Having determined the existence of a murmur, its rhythm, pitch, intensity, and quality, we next determine its point of maximum intensity. These points of maximum intensity for murmurs at the four valvular orifices of the heart may be briefly summarized as follows: Murmurs arising at the mitral valve are loudest at the apex of the heart or immediately above it; tricuspid murmurs are loudest over the lower part of the sternum; pulmonary murmurs, in the second left intercostal space close to the sternum; and aortic murmurs, in the second right intercostal space at the edge of the sternum and over the whole length of the body of that bone.

Valvular diseases which cause murmurs result either in a condition of the valves that allows regurgitation, or one that obstructs the onward blood-current. Valvular insufficiency arises when extensive retraction, perforation, or partial detachment of the valves prevents them from completely closing their respective orifices. And when the chordæ tendineæ have been ruptured, or when calcareous degeneration has made the valves or the parts in the immediate vicinity abnormally rigid, the regurgitant current through the aperture thus left gives rise to a regurgitant murmur.

{654} When the valves are thickened, retracted, adherent, hypertrophied, or degenerated, so that their edges are prevented from being accurately applied to the walls of the ventricles or vessels, they obstruct the current of blood, and the impinging of the blood-current against the obstruction gives rise to obstructive murmurs. These conditions--stenosis and insufficiency--are often found coexisting, but rarely in equal degree, one usually predominating sufficiently over the other as to give a dominant character to the murmur.

The lesions which produce these conditions may be temporary or permanent--temporary when they occur during the course of acute endocarditis, and permanent when they consist of a new growth either of connective, fibroid, calcareous, or atheromatous tissue, which alters the form of the valves and impairs their function. Acute and chronic valvular disease may produce the same murmurs. The effect of the valvular deformity depends entirely upon its seat.

In the study of the relations of valvular lesions to cardiac murmurs physical signs are the important factors in their diagnosis, and it is necessary always to bear in mind the normal physiological conditions which constitute a complete cardiac pulsation.

The apex of the normal heart is felt between the fifth and sixth ribs on the left side, about two inches below the nipple and one inch to its sternal side. The base of the heart is on a level with the third costal cartilages. The tricuspid valve lies behind the middle of the sternum, on a line with the articulations of the cartilages of the fourth ribs with the sternum. The mitral valve lies behind the cartilage of the fourth left rib, near the edge of the sternum. The aortic valves lie behind the sternum, a little below the junction of the cartilages of the third ribs with the sternum, near its left edge. The pulmonary valves lie behind the junction of the third left rib with the sternum.

Let it be remembered that the tricuspid orifice is the most superficial, then the pulmonary, next the aortic, and, deepest of all, the mitral orifice. Ranged from above downward, the pulmonary orifice comes first, then the aortic, then the mitral, and lastly the tricuspid.

Aortic Obstruction, or Stenosis.

Stenosis at the aortic orifice is a common cardiac lesion, and one that is always accompanied by more or less hypertrophy of the left ventricular walls.

MORBID ANATOMY.--In aortic obstruction the cardiac valves will be found to present some or all of the changes which have been described as taking place in the course of acute and interstitial endocarditis, together with degenerative changes due to atheromatous, calcareous, fibroid, fatty, or connective-tissue metamorphosis.

Sometimes the valves may be covered with thick, warty, irregular excrescences that cause loud murmurs, and yet do not seriously interfere with the outgoing blood-current. At other times stenosis of the aortic orifice may be so extensive as to almost obliterate it. When such is the case, the extent of the lesion will be measured much more by the consequent hypertrophy and its effects on the systemic circulation than by the loudness or harshness of the murmur which it produces.

Very frequently the valves are so rigid that they cannot be pressed back against the wall of the aorta, and these unyielding prominences are greater obstacles to the outgoing current of blood than vegetations on the surface of the valves.

In a few rare cases the outlet may be diminished by constriction of the {655} aorta at the point of insertion of the valves. Adhesion of the aortic valves begins at their bases and extends along their free edges to their tips; sometimes they become fused together into a mass, so that they project into the blood-stream in the form of a funnel irregular in shape and studded with calcareous nodules. The line of attachment of the valves to the aorta frequently becomes entirely obliterated.

In some instances the contraction of the valves between their points of attachment causes them to form a deep pocket or pouch, and their points of attachment may be a quarter of an inch apart.

Obstructions at the aortic orifice are frequently accompanied by atheromatous changes in the aorta, the result of chronic inflammation of its tunics--arteritis deformans.

As a result of aortic stenosis the wall of the left ventricle becomes hypertrophied. This change is a gradual one, and is called compensatory hypertrophy: it is due to the increased force required to propel the blood through the constricted orifice.

After a time insufficiency of the mitral valves is apt to occur, caused either by the extension of endocardial inflammation from the aortic valves or by the forcible pressure of blood upon the ventricular surface of the valves.

A slight thickening or roughening of the aortic valves may cause slight obstruction to the outgoing blood-current, which will interfere but little with the emptying of the ventricular cavity, and which rarely leads to hypertrophy of their walls.

ETIOLOGY.--Aortic obstruction is most frequently met with in early and advanced life, the mean age being forty-seven years. It is not uncommon in children; valvular lesions have been found in children under two years of age. It may be induced where the aorta is defectively developed, and some think that imperfect development of the trachea may lead to imperfect expansion of the chest, and thus induce disease of the aortic valve.[6]

[Footnote 6: Barlow in Guy's Hospital Reports, S. 1, vol. vi. p. 235.]

Its most frequent cause is acute exudative and interstitial rheumatic endocarditis. The origin of nearly all valvular disease may be traced back to an attack of rheumatic fever. Next to acute rheumatism, chorea is its most frequent cause. Bright's disease and pyæmia may cause it, and atheroma or arteritis deformans extending to the valves will give rise to valvular lesions which cause obstruction.

Any of the conditions that cause acute exudative and interstitial endocarditis may effect changes in the valves, and the tissue thus developed, undergoing atheromatous, fatty, fibroid, calcareous, or connective-tissue change, will cause obstruction.

Increased tension of the aorta may be the result of chronic spinal deformity, and may be regarded as the indirect cause of aortic stenosis.

The connection between cancer and cardiac valvular disease is to be noticed, if not as cause and effect, at least as a remarkable and noteworthy coincidence.

Women are far less subject to aortic obstruction after rheumatism than men. In men the aortic valves are subject to more pressure and strain than in women, and hence non-rheumatic disease of these valves is very common, while in women it is very rare.

Aortic disease especially occurs in men whose occupations involve repeated, sudden, and great muscular effort.

In old age the walls of the aorta are weakened, and when aortic disease is met with in young subjects it must be regarded as the result of a premature senile condition of the vessels. Allbutt says that in Leeds quite young men have aortic valvular disease, and Peacock mentions several cases where the {656} disease has occurred in young girls who have been placed at service before they were fully developed.

Sometimes the valves are found to be studded with vegetations, apparently of syphilitic origin. Corvisart and Virchow both admit the possibility of such an origin for valvular disease of the heart, but no unquestionable case has as yet been advanced in proof of it. It has been claimed that this is the reason why soldiers so frequently suffer from heart disease; but sailors are notoriously more subject to syphilis than soldiers, and heart disease is rare among them.

The reason is evidently to be found in their mode of dress: sailors wear loose clothes, soldiers have the tightest possible fitting garments. More force is required to pump the blood through the constricted vessels, hence arises more strain on the aorta and more strain on the valves.

Single, sudden muscular efforts have in a limited number of cases produced disease at the aortic orifice.

Aortic valvular disease more frequently than mitral is of non-rheumatic origin; it is slower in its development, and is more commonly met with in advanced life.

SYMPTOMS.--The subjective symptoms of obstruction at the aortic orifice are not usually well marked. Extensive aortic stenosis is not incompatible with a state of comparative good health. As the obstruction to the outflow of blood from the ventricle increases, compensatory ventricular hypertrophy enables the heart to fill the arterial system and relieve the pulmonary pressure. As soon as the ventricular hypertrophy no longer compensates for the obstruction, the arteries are inadequately filled; the left auricle cannot empty itself into the left ventricle, and hence the pulmonary vessels are abnormally full, as is also the entire venous system. The scanty arterial supply gives the pallor to the face which so frequently accompanies this condition, and syncope is liable to occur as a result of partial cerebral anæmia.

These are late effects, and in many cases do not make their appearance until the mitral valve is secondarily involved. The pulse in aortic stenosis is normal in frequency, diminished in volume and power, usually regular in rhythm, though it may be intermittent, and is compressible and jerky in character.

As a general rule, in aortic stenosis signs of arterial anæmia precede evidences of venous engorgement. The obstruction to the exit of blood is shown in the sphygmographic tracing by a slanting or oblique up-stroke, as seen in the accompanying tracing, or, as Mahomed says, "the influence of percussion is lost." Tracings of the pulse in aortic stenosis sometimes show considerable separation between the percussion and the tidal waves. In some rare instances the pulse is slowed. There may be slight palpitation, and pain in the chest may sometimes occur in paroxysms; but pain in the chest is far more common in regurgitation than in obstruction. Aortic obstruction is more frequently connected with cerebral embolism than any other valvular lesion.

The left middle cerebral artery is the most common seat of aortic cardiac emboli. The left lower limb is more subject to embolism from aortic valvular disease than the right. The splenic and renal vessels are also the frequent seat of such emboli. Sometimes embolism is due to small auricular or ventricular {657} clots that form behind the obstruction. Such clots have occluded the aortic orifice and caused sudden death.[7]

[Footnote 7: Pathological Transactions, vol. ix. p. 91.]

PHYSICAL SIGNS.--The physical signs of aortic obstruction are generally distinctive and easily appreciated.

Inspection.--The visible area of the cardiac impulse is abnormally increased. Very extensive increase in the area of impulse is frequently accompanied by a lifting of the chest-wall over the heart.

Palpation.--The impulse is felt to be forcible, and is sometimes accompanied by a heaving or lifting motion. The apex is displaced toward the left and slightly downward. A sensation will sometimes be imparted to the hand during systole similar to that produced on the sense of hearing by the whizzing of a missile by the ear. This is often nothing more than an intensified endocardial thrill. This systolic frémissement radiates to the ensiform process of the sternum, being most intense in the second right intercostal space.

Percussion.--The area of cardiac dulness will be increased in proportion to the displacement of the apex-beat to the left. The increase in dulness measures the amount of left ventricular hypertrophy.

Auscultation.--Aortic stenosis produces a systolic murmur which more frequently accompanies than replaces the first sound of the heart. The maximum intensity of this murmur is usually at the second sterno-costal articulation of the right side, but it may be heard with equal intensity over the whole upper part of the sternum, and followed up the aorta and along the carotids; again, it may be loudest at the xiphoid cartilage, or it may be heard with greatest intensity at the junction of the left third rib with the sternum. In most cases the first sound is heard with the murmur, but the murmur may entirely replace or obscure it. This murmur is usually loud and harsh in character, and is loudest at the beginning of the systole. Harshness is one of its distinguishing characteristics.

In pure aortic stenosis the aortic second sound may be inaudible, and is always feeble, but the pulmonic second sound will always be audible. The area of diffusion of this murmur follows the law that a murmur is propagated in the direction of the blood-current. It is conveyed along the aorta into the carotids, and one of its characteristics is that it is heard in the great vessels of the neck. It may sometimes be heard in the thoracic and abdominal aorta.

When an aortic obstructive murmur is heard at the apex its intensity is diminished, and when heard behind it is most distinct at the left of the third and fourth vertebræ near their spines, and frequently extends downward along the spine in the course of the aorta, but with diminished intensity. It is to be noted here that a systolic murmur, audible at the base, and traceable along the ascending arch toward the end of the right clavicle, is by no means limited to cases of aortic stenosis, although aortic stenosis always produces a murmur with these characteristics.

Arterial murmurs, synchronous with the cardiac systole, are far more frequent than diastolic murmurs. When the mitral or tricuspid valves are thickened or incompetent, or when the myocardium is the seat of extensive fatty degeneration, the murmur of aortic obstruction will entirely replace the first sound of the heart.

DIFFERENTIAL DIAGNOSIS.--Aortic obstruction may be mistaken for mitral regurgitation, tricuspid regurgitation; an anæmic bruit, for the murmur of a thoracic aneurism and for a murmur produced by a scabrous condition of the ascending arch of the aorta.

1. Both mitral and tricuspid regurgitation, as well as aortic stenosis, are recognized by a systolic murmur. The murmur of aortic obstruction is heard with its maximum intensity at the second right sterno-costal articulation, and {658} diminishes in intensity toward the apex. The murmur of mitral regurgitation is heard with greatest intensity at the apex-beat. The murmur of aortic obstruction is conveyed into the vessels of the neck; that of mitral regurgitation to the left, in the direction of the apex-beat, and is heard behind, between the fifth and eighth dorsal vertebræ, at the left of the spine, with very nearly the same intensity as at the apex. The pulse in aortic stenosis is hard, firm, and wiry in character, but regular, while in mitral regurgitation the pulse is irregular in rhythm as well as in force, is never incompressible, and is easily increased in frequency. Gastric, intestinal, renal, hepatic, and bronchial symptoms are present in mitral regurgitation, while the subjective symptoms of aortic obstruction are cerebral in character. The pulmonic second sound is generally feeble in aortic stenosis, while in mitral regurgitation it is intensified. The murmur of aortic stenosis is harsh; the murmur of mitral regurgitation is soft, and frequently musical in character.

2. Tricuspid regurgitation is also accompanied by a systolic murmur. But while the murmur of aortic stenosis has its maximum intensity at the right second sterno-costal articulation, the murmur of tricuspid regurgitation is very rarely heard above the third rib: this is an important diagnostic sign. Tricuspid regurgitation is accompanied by jugular pulsation, while the murmur of aortic obstruction is heard in the arterial trunks of the neck. To distinguish between intrinsic pulsation of the jugular vein and throbbing of the carotid arteries press lightly on the vessel above the clavicle; this arrests pulsation when due to tricuspid disease, while if due to aortic stenosis the result is negative. Moreover, respiration influences jugular pulsation, while it has no influence over carotid throbbing. The area of transmission of tricuspid regurgitant murmurs is not more than two inches from the point of their maximum intensity; whereas the aortic obstructive murmurs are conveyed along the sternum into the vessels of the neck. There is nothing peculiar or abnormal about the pulse of tricuspid regurgitation, while the hard and wiry pulse of aortic obstruction is quite characteristic.

3. An anæmic bruit may be mistaken for aortic stenosis, since the rhythm and seat of the bruit are often identical with those of the stenosis. Anæmia, however, produces a murmur that is heard loudest in the carotids, and is accompanied by a venous hum, the bruit du diable, which is continuous, and heard best on the right side of the neck. Thus in anæmia there are three murmurs, arterial, cardiac, and venous. In aortic disease the murmur has its maximum intensity at the second sterno-costal articulation of the right side, and is not accompanied by a venous hum. There is always more or less cardiac hypertrophy in stenosis, and an increase in the force of the apex-beat, while anæmia is attended by a feeble cardiac impulse. The murmur is soft and blowing in anæmia and harsh and rasping in aortic obstruction. The pulse is characteristic in aortic stenosis; in anæmia it may have a thrill, but is never hard and wiry. Lastly, the subjective signs of anæmia will render the diagnosis comparatively easy, especially when the hum in the veins coexists.

Aortic disease usually occurs in those who have passed middle life as a rule, and in men, while young females are the chief subjects of anæmic murmurs.

4. Thoracic aneurism may produce murmurs resembling those of aortic stenosis. The dilating impulse on palpation, the normal force of the heart-beat, the single or double bruit, the pain,--all these symptoms of thoracic aneurism are absent when aortic stenosis alone is present. Moreover, the history of the case will greatly aid in the diagnosis; and, lastly, aneurismal murmurs have their maximum intensity at the seat of the tumor, and not at the base of the heart.

5. A murmur from a scabrous state of the arch of the aorta is exceedingly {659} rare. It is located higher up than that of aortic stenosis, is not transmitted into the cervical vessels, and has its maximum intensity over the transverse portion of the arch.

Aortic Insufficiency, or Regurgitation.

Aortic insufficiency is an abnormal condition of the aortic valves which prevents their complete closure and allows a backward current of blood to flow from the aorta into the left ventricle during the diastole. This lesion is rarely found unassociated with aortic stenosis, and together they constitute one of the most important and frequent valvular lesions. It is sometimes called aortic incompetence, aortic inadequacy, and aortic reflux.

MORBID ANATOMY.--In a normal heart at diastole the aortic semi-lunar valves are firmly closed, so as to completely fill the orifice between the left ventricle and the aorta. In aortic insufficiency the valves are prevented from performing their normal function, on account of the following anatomical changes. As a result of interstitial endocarditis the valves may have been thickened, puckered, and shortened, so that they do not meet when brought into the plane of the orifice.

When the central portion of the segment is indurated, the whole valve subsequently curls up, either toward the orifice or back against the wall of the aorta, and in either case there is insufficiency of the valves. In the first case there is insufficiency with great obstruction; in the second, with but very slight obstruction.

These processes of thickening and shortening are usually the result of the train of changes which attend and follow endocardial inflammation, but they may also come as the result of an atheromatous process extending from the aorta to the valves; and it may be mentioned here that the atheromatous changes, by impairing the elasticity of the aortic walls, become a source of imperfect coronary circulation, and hence prepare the heart for that dilatation whose other causes will subsequently be described.

Regurgitation may result not so much from shortening as from adhesion of the valve-tips to the walls of the aorta. There may be depression of the valves which comes from over-extension, and then extreme insufficiency will be the result. When this pathological lesion occurs, usually only one segment is involved. Complete retroversion of the valves is a questionable lesion; still, it may occur. Again, one or more segments may be more or less detached from their points of insertion, or from the same causes a valvular aneurism or a diseased valve may be torn or ruptured, and then perforation allows a free opening for the regurgitant passage of the blood.

After extensive obstruction has existed for a long time little tunnels may form by the side of the valves and permit a regurgitant current from the aorta to the ventricle. The aortic valves are more liable to laceration than any other valves. Not infrequently the ragged edge of a lacerated or displaced aortic valve is found covered with fibrinous efflorescences of larger or smaller size.

During a cardiac diastole, normally, the blood is passing from the auricle into an empty ventricle; when, however, regurgitation has persisted for a considerable time, there will be added to the primary stream (which of itself is capable of filling the cavity of the ventricle) a regurgitant stream from the aorta, and by this combination of two streams the left ventricle becomes over-distended and permanently dilated. This dilatation occurs all the more readily since during the diastole the ventricular walls are relaxed and less capable of resisting the increased blood-pressure. Thus, permanent dilatation of the left ventricle occurs in a comparatively short time; and to overcome {660} the dilatation and the obstruction to the cardiac circulation the left ventricular walls hypertrophy. The hypertrophy goes on increasing until it compensates for the dilatation; but before this point is reached the ventricular cavity sometimes becomes very much dilated and the left heart reaches an immense size.

This dilatation and hypertrophy may be so extensive that the organ often weighs twenty or thirty ounces, a case being recorded where the enormous weight of forty-eight ounces was reached.[8] The heart is then frequently called the cor bovinum. In such cases the organ has a peculiar pointed form, the right ventricle appearing like a mere appendix. The left ventricle is thus capable of containing so much blood, and such an abnormally large amount is thrown into the aorta at each cardiac cycle, that the arterial system is largely over-filled. Hence the arteries are elongated during their pulsations more than in health, and often become distinctly flexuous with each cardiac pulsation.

[Footnote 8: See Hilton Fagge, Diseases of the Valves of the Heart.]

The increase in the ventricular power and in the amount of blood contained in the ventricles and thrown against the aortic walls leads to endarteritis and subsequent atheromatous degeneration of the arterial walls, and the arteries become so brittle that during excitement they may suddenly rupture and cerebral apoplexy result; aneurism is also liable to be developed under such conditions.

In the normal heart the aortic recoil is the force which propels the blood into the coronary arteries. When the aortic valves are insufficient, and furnish little or no resistance to the return blood-current, the coronary blood-supply is consequently diminished. When perfect compensation has existed for some time, it begins to fail, and dilatation again commences at the expense of the walls of the heart. This dilatation is aided, first, by the condition of the coronary arteries above referred to, and, secondly, by the fact that aortic recoil is now expended as much in driving a regurgitant current into the ventricle as in forcing blood through the coronary vessels.

In some cases atrophy of the papillary muscles allows the mitral flaps to swing back into the left auricle when increased pressure is exerted upon them. When from any one of these causes mitral incompetence becomes secondary to, and coexistent with, aortic insufficiency, all the signs of impeded venous circulation will be present. These changes will be considered under the head of Mitral Disease.

When over-distension of the left ventricle causes incomplete emptying of the left auricle, a greater or less amount of passive hyperæmia of the lungs may be present without mitral insufficiency.

ETIOLOGY.--The etiology of aortic insufficiency is similar to that of aortic stenosis. Rheumatic endocarditis is undoubtedly its most frequent cause, but it may also have its origin in sudden and violent muscular effort, atheroma of the aorta, endarteritis, congenital malformation, and enlargement of the aortic orifice. Congenital malformation or congenital non-development is, according to Virchow, a frequent cause in chlorotic females.

In many cases the atheroma that causes the incompetence is of gouty origin, especially when gouty kidneys coexist or when alcoholismus is associated with a gouty diathesis.

Sometimes aortic incompetence is the result of imperfect development of the aortic valves. A rare case is recorded in the Pathological Transactions (vol. xvi. p. 77), where a young man fell from a height upon his side and tore off an entire flap of the semi-lunar valve: there was no external mark of injury, and the rupture was plainly due to the transmission of rapid vibrations from the jarred surface. Valvular inadequacy sometimes results from dilatation of the aorta at its origin.

{661} There can be little doubt but that the interstitial inflammation which gives rise to the valvular changes which allow aortic regurgitation is often excited by the violence with which the aortic valves are closed by the backward rush of blood on the aortic recoil during prolonged and violent physical exertion.

Thus, although rheumatism plays a very important part in its development, it is so far from being its sole cause that C. Hilton Fagge says that in at least half the cases of this form of valvular disease met with in London hospitals one fails to elicit a rheumatic history.

SYMPTOMS.--Rational Signs.--So long as hypertrophy of the left ventricle compensates for its dilatation, the individual will suffer little or no inconvenience, even though the regurgitation is extensive. When the regurgitant stream is a very small one there will also be little or no disturbance of the general health.

But the compensation is only maintained for a short time. When the equilibrium is lost the eccentric hypertrophy induces excessive heart-action during mental excitement or violent muscular effort. The action of the heart then becomes labored, and the patient becomes anxious, nervous, and fretful. Sufferers from aortic regurgitation are generally aware that exercise will augment all their uncomfortable symptoms. Their respirations are accelerated by moderate exercise, and are accompanied by cardiac palpitation. As the insufficiency increases attacks of headache and vertigo become more and more prolonged and severe; the patient complains of muscæ volitantes, dyspnoea, giddiness, and is compelled to sleep with his head elevated. Palpitation is now a constant symptom, and a visible carotid impulse is persistently present.

A comparatively frequent symptom of aortic regurgitation is a distinctly paroxysmal shooting or stabbing pain over the heart, in the left shoulder, or extending down the left arm. Sometimes this pain is accompanied by numbness and a peculiar whiteness of the skin along the line of the pain. In other cases the pain passes from the middle of the sternum to the right arm. This pain is increased by mental excitement and muscular exertion, and sometimes by over-distension of the stomach. In a few cases patients will complain of a sickening fluttering of the heart.

When the nutrition of the hypertrophied ventricular walls becomes markedly interfered with, or when insufficiency of the mitral valves occurs, the veins of the systemic circulation become overloaded, as is evidenced by cyanosis and dropsy; the dropsy appears first as oedema of the feet, and gradually extends upward until a condition of general anasarca is reached. The cyanosis is increased after slight exertion, and is accompanied by dyspnoea, carotid pulsation, and puffiness of the face.

In the advanced stages of the disease there is orthopnoea, sudden starting in sleep, angina pectoris, and in some cases albuminuria and enlargement and tenderness of the liver. Attacks of syncope at first occur only after active muscular exercise, but later they occur independently of it, and are extremely distressing. These patients are in danger of death at any moment, either during a state of the utmost calm or the most intense excitement; the danger is greater, however, during exertion.

The pulse is the most characteristic subjective symptom of this form of valvular lesion. It was first accurately described by Sir Dominick Corrigan,[9] and it is frequently called Corrigan's pulse. He especially said that the disease was indicated by visible pulsation of the vessels of the head, neck, and upper extremities. On account of the elongation of the arteries during their pulsation, and their becoming distinctly flexuous, the pulse is frequently called the locomotive pulse. It is large and distinct, rapidly projected against the finger, and just as quickly the arterial tension sinks to its minimum and the {662} impulse vanishes. It is sometimes accompanied by a vibrating jar, on account of which it is called the water-hammer, jerking, splashing, or collapsing pulse. Its characteristics are more apparent when the arm is raised above the head. Although slightly infrequent, quick, and jerking, it is always regular in rhythm; the radial impulse is felt a little after the apex-beat. Thus the pulse-wave of aortic regurgitation travels slowly along the arteries. This delay in the pulse is constant.

[Footnote 9: Edin. Med. and Surq. Journ., April, 1832.]

As soon as the systemic circulation is overloaded from insufficiency of the heart or from secondary mitral insufficiency, the pulse becomes feeble and irregular upon the slightest exertion, and may intermit, but it is still of the same peculiar jerking character. The sphygmographic tracings of this pulse show a high upstroke and absence of the dicrotic wave.

This vibrating pulse or pulse of unfilled arteries is usually possessed of fulness of volume, but when obstruction coexists it may be small and flickering unless the arteries are calcified or atheromatous. The pulse of aortic insufficiency taken by the sphygmograph resembles strongly the pouls des vieillards, but the senile pulse gives a rounded instead of a pointed summit. Still, in old age the two tracings may be indistinguishable.[10] The peculiar crochet or beak is noticeable in graphic tracings of the pulse of aortic inadequacy.

[Footnote 10: Marey, Phys. Méd. de Circ. du Sang, Paris, 1863.]

Stokes has described, under the designation of steel-hammer pulse, a peculiar and characteristic pulsation of the arteries which occurs in cases of acute rheumatic arthritis supervening upon chronic inadequacy of the aortic valves. The pulse is abrupt and energetic, as the rebound of a smith's hammer from the anvil; it is exhibited, however, only in the arteries adjacent to the affected joints.[11]

[Footnote 11: Continued Fever, 1874, p. 244.]

PHYSICAL SIGNS.--Inspection.--There is an increase in the area of the apex-beat, which is plainly more forcible and is visible over a wider area than in aortic obstruction. After compensation has ceased to balance the forces in the heart the apex-beat becomes more and more feeble and diffused. One of the most important points obtained by inspection is pulsation of the carotids and the vessels of the upper extremities. Becker and Quincke have observed pulsation of the retinal vessels in cases of extensive aortic regurgitation.[12]

[Footnote 12: London Ophth. Hosp. Rep., Feb., 1873.]

Palpation.--On placing the hand over the præcordial region a heaving, lifting impulse will be perceived, which is transmitted over a large portion of the thoracic walls. The apex-beat is displaced downward and toward the left, sometimes as far as the eighth rib and two and a half inches to the left of the left nipple. Occasionally a continuous diastolic thrill, equally intense during the whole of the diastole, is felt over the sternum, most distinctly at the site of the aortic valves. In some cases there is a slight pulsation in the scrobiculus cordis.

{663} Percussion.--The area of percussion dulness corresponds to the extent of the cardiac enlargement. Deep dulness is elicited below and to the left of the normal area, and its outline has more of an oval contour than in health. So soon as the cardiac dilatation exceeds the hypertrophy, the area of dulness will extend horizontally rather than vertically, and it may be carried slightly upward, the apex beating in the axillary space. The area of dulness may extend six and a half inches from right to left, and from the upper edge of the third rib to the line of the liver dulness. The superficial area of dulness is likewise increased horizontally and toward the left.

Auscultation.--Aortic regurgitation is characterized by a diastolic murmur, which may take the place of, or immediately follow, the second sound of the heart. It is very distinct at any point over the base of the heart, but usually has its maximum intensity either at the sternal end of the second right costal cartilage, in the second right intercostal space, or at the sternal junction of the third rib on the left side. It is transmitted over the sternum, and sometimes will be loudest at the xiphoid cartilage, and is thence transmitted in the direction of the apex. Its area of diffusion is greater than that of any other cardiac murmur: it is not only conducted down the sternum to the xiphoid cartilage and to the apex, but it may be heard at the sides of the chest along the spinal column, and sometimes faintly in the ascending and transverse portions of the arch, in the carotids, and in rare instances as far as the radial arteries. The murmur of aortic reflux is accompanying rather than substitutive, for the pulmonic second sound is audible at the right base.

Foster[13] regards incompetency of the posterior segment of the valve as producing a murmur which is conducted to the apex, whereas inadequacy of either or both of the anterior segments is accompanied by a murmur which is conducted to the ensiform cartilage. This point has a practical bearing on account of the relationship of the anterior segments of the valve to the coronary arteries. If the murmur indicates a lesion of the posterior flap of the valve, the prognosis will be better. When the second sound of the heart is distinct the murmur immediately follows it. Many English writers call the murmur a post-diastolic aortic murmur.

[Footnote 13: Med. Times and Gaz., 1873, vol. ii. p. 658 et seq.]

Although having the greatest area of diffusion, aortic reflux has not the loudest murmur; it is soft, blowing, sometimes rough, and frequently musical. It is loudest at the beginning of diastole, gradually decreasing in intensity, although it may preserve its rushing, blowing character during all the diastole.

An aortic regurgitant murmur may temporarily disappear if a plug of fibrin closes the orifice, or if the walls of the left ventricle are the seat of extensive fatty degeneration, the aorta being rigid and inelastic.[14] When aortic stenosis coexists there will be a double murmur, audible over a very large space, having its maximum intensity at the right edge of the sternum in the second interspace.

[Footnote 14: Brit. Med. Journ., 30th March, 1882.]

Systolic and diastolic murmurs, though sometimes separated by a well-defined pause, may run into each other. If mitral regurgitation occurs with aortic regurgitation, each murmur retains its own location of maximum intensity. In rare instances, when two segments of the valve are healthy, a clear aortic second sound is heard, which is preceded by a faint reflux murmur. Such a murmur is said to be prediastolic in rhythm. Aortic reflux murmurs are often very indistinct, and can only be heard when the patient is in the recumbent posture. There is no necessary connection between the amount of reflux and the loudness of the murmur.

A diastolic murmur heard at or below the level of the aortic valves, chiefly {664} audible in the line of the sternum, indicates considerable aortic incompetence. If a diastolic murmur is inaudible in the carotids, it is usually preceded by a systolic murmur, which has its maximum intensity at the aortic valves or in the so-called aortic area: such a murmur indicates comparatively trifling incompetence with considerable obstruction, probably produced by calcified semi-lunar valves.

If a diastolic murmur is distinctly audible in the carotid arteries, it is invariably preceded by a loud systolic murmur in them, the systolic portion of the murmur not being very plainly audible in the aortic nor in any part of the cardiac area: this indicates very considerable incompetence with comparatively trifling obstruction.

DIFFERENTIAL DIAGNOSIS.--The diagnosis of aortic regurgitation is generally not difficult, as it rests almost exclusively upon the existence or nonexistence of a diastolic murmur. It may, however, be mistaken for aortic stenosis, for mitral obstruction, for pericarditis localized over the aorta, for aneurism of the aorta, for aneurism of the aorta immediately above the valves, patency of the ductus arteriosus, for insufficiency of the pulmonic semi-lunar valves, and, occasionally, for a rough and inelastic condition of the ascending aorta.

1st. Mitral obstruction gives a presystolic murmur, while aortic reflux produces a diastolic murmur. Mitral stenosis is accompanied by no hypertrophy or dilatation of the left ventricle, whereas these conditions are always present with aortic reflux. The quality of a presystolic mitral murmur is harsh and rough, and it has a churning, blubbering, or grinding character, while aortic reflux has a murmur of low pitch and of a soft, blowing, or musical character. Mitral stenosis is accompanied by a purring thrill which is absent in aortic regurgitation. The murmur of mitral stenosis is the longest of all the cardiac murmurs. The murmur of mitral stenosis is never heard behind, whereas that of aortic regurgitation is often heard at the sides of the chest and along the spinal column. Finally, mitral stenosis is attended by well-marked pulmonary symptoms during active physical exertion, which are rarely present in aortic insufficiency.

2d. A pericardial friction sound over the aorta has its maximum intensity over the seat of its production, and is usually audible during both the cardiac systole and diastole. In aortic regurgitation the character of the pulse, the existence of hypertrophy and dilatation of the left ventricle, and the carotid pulsation will establish the diagnosis.

3d. An aneurism at the sinuses of Valsalva is diagnosticated by the history of the case, the presence of the murmur over the pulmonary artery, the evidences of arterial degeneration, the absence of left ventricular dilatation and hypertrophy, and the peculiar jerking pulse. An aneurismal murmur is circumscribed, has a booming quality, and is usually systolic in rhythm and never transmitted to the apex of the heart.

4th. Patency of the ductus arteriosus is a rare condition: in a case where this was diagnosticated[15] the murmur was audible at the left of the sternum, was not everywhere continuous with the second sound, was only transmitted very feebly to the left, and had a wavy character, sufficient of itself to distinguish it from an aortic regurgitant murmur.

[Footnote 15: Guy's Hosp. Rep., Ser. 3, vol. xviii., 1872-73.]

5th. Insufficiency of the pulmonic semi-lunar valves is the rarest of all valvular lesions: the murmur should be diastolic, having its maximum intensity in the second intercostal space of the left side; it should be transmitted only downward and toward the right apex; and should not be attended by arterial pulsation, a jerking pulse, or by left ventricular hypertrophy and dilatation.

6th. A diastolic murmur in the ascending arch, due to roughening, rigidity, {665} and dilatation of the artery, is also rare, while the condition which some say can produce it is very common.

Two cases are recorded in which the diagnosis rested upon the character of the pulse, throbbing of the arteries, and the absence of hypertrophy and dilatation of the left ventricle.[16]

[Footnote 16: Bellingham Dis. of Heart, 1857, p. 152; also Trans. Path. Society, vol. iii., March, 1868, p. 3, article by Prof. Law.]

Mitral Stenosis.

Stenosis, or obstruction of the auriculo-ventricular opening of the left heart, is due partially to constriction at the base of the mitral valves, and partially to adhesions of the valve-tips or chordæ tendineæ. It usually occurs as a consequence of rheumatic endocarditis, rarely of atheromatous degeneration, and is most likely to occur in endocarditis affecting young persons.

Mitral disease is present in one-half the cases of valvular diseases of the heart. Usually, insufficiency and stenosis of the mitral orifice occur together, and stenosis probably never occurs without some insufficiency.

MORBID ANATOMY.--As a result of acute exudative or interstitial endocarditis, the valves are rendered shorter and narrower, as well as thicker and more cartilaginous, than normal. These rigid valvular projections not only obstruct the flow of blood from the auricle to the ventricle, but allow of its regurgitation from the ventricle into the auricle. In mitral stenosis there is not only thickening and contraction of the valves, but the valve-tips or the chordæ tendineæ become adherent, and sometimes each papillary muscle gives rise to a corrugated, cylindrical mass pierced with one or more slits, indicating the chordæ of which it was originally made up. The wall of the valve, especially toward its free edge, is greatly thickened, and these thickened portions are so dense that they have a distinctly cartilaginous feel. On the valvular flaps that have undergone this sclerotic change calcareous masses are very frequently developed, and are especially liable to form when a gouty diathesis exists.

When the chordæ tendineæ and papillary muscles have become adherent, the edges of the valves are drawn down toward the apex of the heart; and since the flaps are adherent for a greater or less distance upward from their bases, the valve presents a funnel-shaped appearance with its base looking toward the auricle and its apex toward the ventricle, whose smaller opening, rarely circular, usually resembles a slit with its axis in the line which unites the original segments of the valve. This button-hole slit may scarcely admit the tip of the little finger, while the normal mitral orifice permits the easy introduction of three fingers.

Annular (ring-like) stenosis is far more common at the mitral than at the aortic orifice. Hard, wart-like vegetations frequently develop on the puckered and seamed flaps, which increase the already existing obstruction. Sometimes the funnel-shaped appearance is wanting, and the flaps are stretched horizontally across, with a small opening in the centre, like a diaphragm: looked at it from the auricle, this slit is often crescentic in shape.

In cases of long standing the vegetations may become calcified. If the new tissue in the diseased valves undergoes fatty change and softens, ulcerative processes are set up and the chordæ tendineæ may rupture. On the floor of such ulcers calcareous masses and débris are frequently found.

Hayden thinks that "all funnel-shaped mitral stenosis is the result of primary acute inflammation and thickening of the valve-segments, with cohesion of their adjacent edges." Out of 62 cases of mitral stenosis, 59 assumed the button-hole form, and 3 only the funnel-shape (Fagge and Hayden).

{666} In rare instances the tendons will adhere to the wall of the heart as well as become matted together. Adjacent to the valves the endocardium will usually be found slightly thickened.

The following changes are developed in the heart and vessels as the result of mitral stenosis: The left ventricle becomes smaller, and sometimes its walls are thinner than normal. The aorta is also small and thin-walled. An almost necessary result of mitral stenosis is dilatation, with subsequent hypertrophy of the left auricle. Sometimes the auricular cavity is enormously dilated--so much so that fifty years ago Thurman described it as true aneurism of the left auricle.[17] Not infrequently the left auricular walls are from one-eighth to one-fourth of an inch in thickness. Its appendix is elongated, assuming a peculiar curved form, the aperture between it and the auricle becoming wider than normal. Moxon records a case of extensive mitral stenosis where the appendix was two and three-quarter inches long.

[Footnote 17: Med.-Chir. Trans., vol. iii., Ser. 2, p. 244.]

As soon as the auricular hypertrophy ceases to be compensatory and dilatation begins, the pulmonary circulation becomes obstructed, causing increased tension in and distension of the pulmonary vessels. The walls of the pulmonary vessels, especially those of the main trunk, are thickened and hypertrophied; in rare cases they have been found twice the thickness of those of the aorta.

Although mitral stenosis is a disease of youth, and atheroma one of old age, yet it not infrequently happens that even before the age of puberty atheromatous degeneration occurs in the pulmonary vessels, especially in the small branches, as a result of the increased blood-tension in the pulmonary system.[18]

[Footnote 18: Trans. Path. Society, vol. xvii. p. 90.]

The passive pulmonary hyperæmia which results from the obstructed pulmonary circulation may lead to those changes which collectively constitute brown induration of the lung. Another occasional occurrence, directly due to extensive mitral stenosis, is nodular hemorrhagic infarction. Hemorrhagic infarction of the lungs is in nearly every case preceded by thrombosis of the right side of the heart.

In some instances the enormously dilated left auricle may, by pressing on a bronchus, reduce its calibre one-half, and thus interfere with the functional activity of the left lung. When the pulmonary hyperæmia is extensive violent physical exertion or violent coughing may cause a rupture of one of the larger pulmonary vessels, and true pulmonary apoplexy result.

Bronchorrhoeal expectoration of large quantities of glairy mucus is a very frequent result of the intense hyperæmia of the mucous membrane of the bronchial tubes which sometimes occurs in mitral stenosis. The secretion is increased with every increase in the passive hyperæmia. The lungs are at all times so liable to congestion and oedema that any sudden or violent exercise may lead to a rapidly fatal result. Again, when the conditions enumerated have existed for some time, mitral stenosis may lead to hypertrophy of the right heart. In some rare cases the tricuspid orifice has become slightly insufficient.

ETIOLOGY.--Mitral disease is especially met with in the young, and in the child it is almost invariably a stenosis. The average age is about thirty-one; it is very rare to find it occurring after the fiftieth year of life. It seems from statistics that it is nearly twice as frequent in females as in males.

It is not infrequently of congenital origin. Acute rheumatic endocarditis is its most frequent cause. The mitral valves are more frequently affected in chorea than the aortic. In some few instances stenosis results from extension of the inflammatory process from the aortic semi-lunar valves, or prolonged aortic regurgitation and stenosis may lead mechanically to mitral disease, but not to stenosis. Niemeyer regards atheroma as an exceptional cause of mitral stenosis. No other authority regards it as a possible cause.

{667} It is a question whether scarlatina or diphtheria tends to produce in children a valvular endocarditis which is followed by mitral stenosis. It seems plausible, since in many young children it is certain that mitral stenosis has not resulted from either rheumatism or chorea. Finally, with the exception of atheroma, all the causes enumerated in the etiology of aortic stenosis may be the cause of mitral stenosis.

SYMPTOMS.--Rational Signs.--The subjective cardiac symptoms of mitral stenosis are few. There may be no such symptoms. Usually, after violent exercise there is more or less cardiac palpitation, but this will cease as soon as the auricle can relieve itself, which is readily accomplished by the patient's assuming a recumbent position on the right side with the head slightly elevated. This class of patients as a rule are pale and anæmic. There is a sharp pain frequently felt in the region of the apex, which is always suggestive of mitral stenosis. The pulse is regular and normal in character so long as the auricular hypertrophy compensates for the auricular dilatation.

When the ventricle is unable to receive and discharge its normal quantity of blood with normal regularity, the pulse becomes small in volume, feeble in force, rapid and irregular in rhythm. The sphygmograph exhibits a tracing, frequently called the mitral pulse; the sphygmograph tracing is the same as when the ventricle throws a greatly diminished blood-current into the aorta (Fig. 44). This is asystolism, and the pulse is a clear indication of the condition.

Balfour differs from other authorities in the statement that among the most remarkable subsidiary phenomena of mitral stenosis is irregularity of cardiac rhythm, which, always present in a greater or less degree, is sometimes a diagnostic phenomenon. The auricular systole commences earlier than normal on account of its hypertrophy. This premature contraction of the auricle, stimulating ventricular contraction, is indicated by a second ventricular systole which is much less forcible than the first.

The passive pulmonary hyperæmia attending the advanced stages of this form of cardiac disease causes habitual dyspnoea, which is exaggerated by physical exertion and is attended by a dry, hacking, teasing cough which resembles the so-called nervous cough.

After violent or prolonged exertion there may be bronchorrhoea, a pint of glairy, watery mucus often being expectorated in a few moments. Not infrequently severe exercise induces attacks of profuse, watery, blood-stained expectoration, indicative of pulmonary congestion and oedema. Sometimes the exertion of walking rapidly against a strong wind will induce such intense congestion and oedema of the lungs in one with extensive mitral stenosis as to cause sudden death.

Hæmoptysis is not infrequent, small quantities of pure florid blood being expectorated.

Orthopnoea is not a frequent symptom of mitral stenosis, for even in extensive and long-standing cases the pulmonary congestion is not constant, as the auricle is able ordinarily to empty itself, and only becomes engorged during active physical exertion or great mental excitement.

It should be mentioned here that the old idea, that "mitral stenosis sometimes produces hypertrophy of the left ventricle," is fallacious. In no instance can it be attributable to mitral stenosis.

{668} Physical Signs.--Inspection.--As the left ventricle does not receive its normal quantity of blood, the cardiac impulse is feeble. Sometimes it has a visible undulating movement.

Palpation.--On palpation, although the apex-beat is less forcible than normal, a distinct purring thrill will be communicated to the hand: this thrill is a constant attendant of mitral stenosis, and may be regarded as its diagnostic sign. It should be remembered, however, that a purring thrill does not always indicate mitral stenosis. It is most distinct at the apex-beat, although it may be diffused over the whole præcordial space. It either continues through the entire diastole or is only present just before the systole. It is sometimes called a presystolic thrill. It ceases with the apex-beat. The only conditions besides mitral stenosis which will cause a purring thrill at the cardiac apex are mitral regurgitation, with extensive dilatation of the left ventricle, and left ventricular aneurism; in both instances the thrill will not be presystolic, but systolic.

Percussion.--The increased size of the left auricle may cause an increase in the area of cardiac dulness upward and to the left at the inner part of the second left interspace. This increased area of dulness will only be recognized on careful percussion during expiration.

Auscultation.--Mitral stenosis is characterized by a loud churning, grinding, or blubbering presystolic murmur; this murmur is of longer duration than any other cardiac murmur, on account of the time required for the blood to pass through the narrowed and obstructed orifice. It ends with the commencement of the first sound and the apex-beat, being synchronous with the purring thrill. The murmur is heard with its maximum intensity a little above the apex-beat.

Cryan records a case where the murmur was absent, but the diagnosis of mitral stenosis was made from the other symptoms. At the autopsy the orifice would barely admit the tip of the little finger, and the absence of the murmur was accounted for by the smallness of the aperture.[19]

[Footnote 19: Trans. Path. Society, Dublin, Part 2, vol. iv., 1870.]

As a rule, mitral stenosis is accompanied by the loudest as well as the longest cardiac murmur. The murmur is always louder when the patient is erect than when in the recumbent posture. For a few days before death, and at any time when there is great constitutional debility, the murmur may be held in abeyance. A presystolic murmur is never present when auriculo-ventricular narrowing does not exist. When this lesion does exist it is never permanently, and very seldom temporarily, absent. A prolonged murmur and a sharp first sound indicate a funnel-shaped stenosis. A murmur immediately following the second sound, and running through the apex-beat, indicates great contraction of the orifice--diaphragmatic contraction. The murmur of mitral stenosis is very rarely, if ever, conveyed to the left of the apex-beat, and it is rarely heard more than two inches to the right of the apex. The second sound of the heart is intensified over the pulmonary valves. When mitral reflux and mitral obstruction coexist, the two murmurs run into each other, constituting a single murmur that may be mistaken for a systolic murmur. The harsh character of the presystolic element of the murmur can always be recognized.

A mitral obstructive murmur is never soft or musical, but there is a rare form of presystolic mitral which is so short as to resemble a tone. A mitral stenotic murmur does not often merge into the first sound of the heart, but is usually separated from it by a short interval. Sometimes a stenotic murmur only becomes audible when the patient sits up. In about one-third of all cases of stenosis of the mitral orifice the second sound is reduplicated. It is best heard at the apex and when the heart's action is slow. The reduplication may be temporarily absent. Pulmonary congestion efficiently accounts for {669} this reduplication. Geigel ascribes it to "non-coincidence in the closure of the valves." Guttman regards it as originating at the stenotic orifice itself. Balfour thinks that thrill and reduplication of the second sound are sufficient to make a diagnosis in the absence of murmur. Some regard the length of the pause between the murmur and the first sound as a measure of the stenosis--the shorter the pause, the greater the stenosis.

DIFFERENTIAL DIAGNOSIS.--The diagnosis of mitral stenosis is not difficult; it mainly depends upon the existence of two physical signs--the purring thrill and a loud, long, blubbering presystolic murmur.

Mitral obstruction may be mistaken for the murmur of aortic regurgitation (see page 657), for a pericardial friction located over the apex, for a prolonged systolic murmur replacing the first sound at the apex, and for a prediastolic basic murmur transmitted to the apex.

1. To diagnosticate between local pericarditis and mitral stenosis, the same methods are employed and the same rules are to be observed as were mentioned in the diagnosis between aortic reflux and local pericarditis (p. 664).

2. A prolonged systolic apexial murmur, enduring as it does for the period of the first sound, that of the short pause, and reaching the second sound, is often accompanied by a muffled second sound readily mistaken for the first. The diagnosis of this murmur rests upon its soft and blowing character, the synchronism of the murmur with the systolic impulse and carotid pulsation, and the fact that there is no murmur with the second sound at the base.

A prediastolic murmur is distinguished from a mitral stenotic murmur by its progressively diminishing intensity from the base to the apex, by its being accompanied by hypertrophy of the left ventricle, and by a jerking, irregular pulse. The preceding tracings explain themselves.

Mitral Regurgitation.

Regurgitation at the mitral orifice is due to a condition of the mitral valves which allows the blood to flow back from the left ventricle into the left auricle. The backward effects of mitral reflux are more varied than those of any other valvular lesion.

It is a common form of valvular disease, and in the majority of cases is the result of acute exudative or interstitial endocarditis.

MORBID ANATOMY.--The most common lesions which give rise to mitral regurgitation are thickening, induration, and shortening of the mitral valves. In rare instances it may occur independent of valvular disease from displacement of one or more of the segments of the valve, the result of changes in the papillary muscles, chordæ tendineæ, or the ventricular walls. It may also occur in extreme anæmia, or from relaxation of the papillary muscles and dilatation of the left ventricle, without a corresponding elongation of the papillary muscles, and from rupture of the chordæ tendineæ. In most instances, however, the valves are shortened, thickened, and indurated.

In some instances lime salts and large masses of chalky matter are found {670} imbedded in the indurated valves. In such cases the surface and edges of the valves are so rough and jagged that more or less obstruction accompanies the regurgitation.

All these changes, except calcification, may also occur in the chordæ tendineæ and columnæ carneæ. The valves may also become adherent to the walls of the ventricles, or as a result of the shrinking and shortening of the chordæ tendineæ the valve-flaps may not pass back to the plane of the orifice.

Again, the valves or the chordæ tendineæ may be ruptured, so that the valves are pressed during the cardiac systole back into the auricle. If the chordæ tendineæ which are inserted nearest the centre of the valve become lengthened, that part of the flap will be bent upon itself, having evidently yielded to the blood-pressure, and this allows of regurgitation. Sometimes, when the valves appear perfectly healthy, by the application of the water test they will be found to be insufficient.

The first effect of mitral regurgitation is dilatation of the left auricle, due to the pressure of the two blood-currents during its diastole--one from the lungs, and the other from the left ventricle. This dilatation leads to thickening and hypertrophy of the left auricular walls. Following this, the pulmonary circulation is impeded, the pulmonary vessels enlarge, and they may undergo degeneration as a result of the continued regurgitant pressure.

Passive congestion of the lungs with brown or pigment induration is an early pathological sequel of mitral regurgitation. The constant interference with the return circulation from the lungs obstructs more or less the outward current of blood to the lungs from the right ventricle. As the obstruction is a gradual one, the right ventricle becomes so hypertrophied as to overcome it. Consequently, the hypertrophied right ventricle compensates at first for the mitral regurgitation, and as long as the right ventricle is able to fully overcome the abnormal pressure of the blood in the lungs from the mitral regurgitation, so long the patients are comfortable. Sooner or later, however, the compensatory hypertrophy of the right ventricle ceases, and a secondary dilatation occurs which admits of no compensation.

This final dilatation of the right ventricle is favored by the myocardial degeneration, which occurs as a result of defective nutrition of the heart-walls; when this condition is reached the veins throughout the body are placed in a similar condition to those in the lungs.

This general venous congestion is indicated by passive hyperæmia of the abdominal viscera and by cyanosis of the surface during active physical exercise.

The liver is the organ first affected, on account of its great vascularity and from the fact that the hepatic veins do not collapse readily and possess no valves. Thus the liver becomes enlarged and stony (the nutmeg liver) as a result of the obstruction to the emptying of the hepatic vein, and when there is coexistent obstruction of the bile-ducts jaundice will be present.

This portal obstruction induces passive hyperæmia of the intestines and stomach, enlargement of the spleen, and large and painful hemorrhoidal tumors. The impediment to the return of blood from the brain causes cerebral congestion; from the kidney, renal congestion; and, finally, the obstruction to the systemic venous return leads to the accumulation of fluid in the areolar tissue and in the cavities. This dropsy generally begins in the feet and extends upward. In females the obstruction in the vena cava inferior induces derangements of the menstrual functions. Ascites, hydrothorax, hydro-pericardium, and pulmonary oedema may subsequently develop.

In addition to these changes, the dilated and hypertrophied left auricle throws an abnormal quantity of blood with abnormal force into the left ventricle during its diastole, which leads to dilatation of its cavity and necessitates a compensatory hypertrophy of the left ventricular walls. This {671} hypertrophy of the left ventricle increases the force of the reflux current, so that during excitement and active physical exertion pulmonary congestion, oedema, and cerebral apoplexy are liable to occur. In many cases of mitral regurgitation, when the venous engorgement is excessive, general dropsy is favored by the anæmia produced by the obstruction of the thoracic duct.

Friedreich maintains that the augmented tension in the venous system causes an increased resistance in the systemic arteries, which leads to left ventricular hypertrophy.

ETIOLOGY.--Mitral regurgitation may occur at any age; it is especially liable to follow rheumatic endocarditis in the young.

Acute exudative and interstitial endocarditis of rheumatic origin is the primary cause of most of the changes which lead to mitral insufficiency. These changes cause the extensive retractions and thickenings which are present in most cases.

It may occur in conditions of extreme anæmia or where there is degeneration of the walls of the left ventricle.

It is not infrequently secondary to changes at the aortic orifice, produced either by an extension of endocarditis from the aortic to the mitral valves and their appendages, or by the secondary mitral valvulitis excited by regurgitant blood-currents from the aorta.

Mitral insufficiency may also be the result of the enlargement of the left auriculo-ventricular orifice which accompanies excessive dilatation of the left ventricle.

Disease of the columnæ carneæ and chordæ tendineæ, when their structures are so weakened as to allow the flaps of the valve to pass back of the plane of the orifice, will also cause mitral insufficiency.

Ulcerative endocarditis may cause it, either by perforation and rupture of the valves or by rupture of the chordæ tendineæ.

SYMPTOMS.--During the early stage of mitral insufficiency, when the hypertrophy of the right ventricle compensates for the regurgitation, there are no rational symptoms which would lead one to suspect its existence; but when the right ventricle is unable to overcome the obstruction to the pulmonary circulation caused by the regurgitant blood-current, there will be more or less dyspnoea, accompanied by a short, hacking cough, with an abundant expectoration of frothy serum. Sometimes the watery expectoration is blood-stained.

Frequently, the blood-stained expectoration is accompanied by free hæmoptysis, although it should be remembered that profuse hæmoptysis is far more frequent with stenosis than with regurgitation at the mitral orifice. But a cough and watery expectoration with occasional dark blood-stains are usually present as an advanced symptom of mitral regurgitation. Active physical exertion increases the dyspnoea and causes cardiac palpitation.

In advanced cases the extremities, face, and lips become blue, the result of the interference with the capillary circulation, and the liver becomes enlarged and hardened--conditions easily recognized by palpation and percussion.

The patient will complain of a sense of weight and fulness in the right hypochondrium, and there will be anorexia, nausea, and a sense of oppression in the epigastrium. Sometimes the hepatic circulation becomes so obstructed that the biliary secretion is interfered with, and jaundice will be added to the cyanotic discoloration, which gives to the surface a peculiar greenish hue.

Following the hepatic derangement are frequent attacks of gastric and intestinal catarrh and evidences of embarrassed renal circulation.

The urine is diminished in quantity, high-colored, and loaded with lithates. Sometimes albumen and fibrinous or blood casts are found in it.

{672} Headache, dizziness, vertigo, stupor, somnolence, and sometimes a peculiar form of delirium of short duration, result from the passive cerebral hyperæmia induced by obstruction in the superior vena cava.

A late symptom of mitral regurgitation is dropsy, which results both from impaired general nutrition and the abnormal blood-pressure in the venous system, both together causing an exudation of the watery portion of the blood through the walls of the vessels. Dropsy, from mechanical causes having their seat in the heart, first appears in the lower extremities, the ankles becoming oedematous, and thence may extend over the whole body. For this condition to be reached it may require several years or only a few months, depending upon the general condition of the patient and the amount of the reflux. With the general anasarca the dyspnoea becomes extreme; the serous cavities of the body as well as the lungs become oedematous; erythema may occur in the region of the groins, the skin exhibiting a tendency to diffuse gangrene.

Late in the disease pulmonary hemorrhagic infarction may occur as a result of metastasis, and this, in the vast majority of cases, lights up a rapidly fatal pneumonia.

All these changes, however grave and urgent they may be, are gradual in their development, so that the condition of the patient is not so insufferable as its description would lead one to suppose.

The pulse of mitral regurgitation is at first in no respect characteristic. It remains regular in force and rhythm, but later it becomes somewhat diminished in force and volume, irregular in its rhythm, and increased in frequency, but never jerking in character. This tracing illustrates my meaning. While it remains full it is feeble and always compressible. When the heart's action is excited, it has a certain tremulousness: these last-named characteristics are to be regarded more as the result of the failure of the left ventricle than of changes in the valvular insufficiency. If a mitral regurgitant pulse has any distinctive peculiarity, it is its diminution in volume.

Coincident mitral or aortic stenosis may render the pulse regular even in extensive mitral regurgitation.

Physical Signs.--Inspection.--The area of visible cardiac impulse extends over an abnormal space, and is more or less distinct according as the right ventricular hypertrophy is moderate or extensive. Sometimes the thoracic wall is seen to rise and fall with each cardiac cycle, and not infrequently the epigastrium exhibits slight pulsation corresponding in rhythm with the heart-beats.

The epigastric pulsation is due to the right ventricular hypertrophy always found with extensive mitral regurgitation.

Skoda, Bamberger, and Leyden record a few instances in which inspection revealed a double impulse accompanying, with more or less regularity, each cardiac systole. This double impulse only occurs in aggravated cases of {673} mitral insufficiency, and arises from non-coincidence of contraction of the two ventricles.

The jugular veins appear swollen, and this is always most conspicuous when the patient is lying down.

Palpation.--The apex-beat is displaced to the left. When hypertrophy predominates over dilatation, the apex-beat is felt lower than normal. When the dilatation exceeds the hypertrophy, the apex-beat is carried outward and often slightly upward. The impulse is diffused and more or less forcible according as the right or left ventricular hypertrophy predominates. This systolic frémissement is most noticeable when the base of the heart lies close to the chest-wall from retraction of the margin of the left lung.

Purring tremor, systolic in rhythm, felt most intensely at the apex and becoming feebler the farther the hand is removed from that part, either to the right or upward, is invariably due to mitral reflux.

Hayden says that it is exceptional to have a purring thrill with simple mitral reflux. I have never found it except in those cases where left ventricular dilatation greatly exceeded the hypertrophy.

Percussion.--Percussion reveals an increase in the area of cardiac dulness, especially laterally; it extends both to the left and right of the normal line, as well as downward. The area of superficial as well as deep-seated dulness will be increased laterally and downward.

Auscultation.--Mitral insufficiency is attended by a systolic murmur which either completely or partially replaces the first sound of the heart. The quality of the murmur is variable, and not in itself as distinctive as that of mitral stenosis. It is usually a soft and blowing bellows murmur; sometimes, toward its end, the murmur will assume a distinctly musical character.

While the first sound of the heart may be heard distinctly in the early stages of mitral reflux, later the murmur in nearly all cases takes the place of the heart-sounds. Hence many English writers rightly denominate this murmur as post-systolic rather than systolic in its nascent stages. It is heard with its maximum intensity at the apex-beat. Its area of diffusion is to the left on a line corresponding to the apex-beat. It is audible at or near the inferior angle of the left scapula. It can be heard between the lower border of the fifth and the upper border of the eighth vertebra, at the left of the spine, with nearly the same intensity as at the apex. The murmur may be absent from the latter situation until cardiac hypertrophy is developed.

The second sound of the heart over the pulmonary valves is accentuated, while below the junction of the third rib with the sternum on the left side both heart-sounds are feeble. Skoda first drew attention to exaggeration of the second pulmonary arterial sound as a positive and unerring indication of mitral regurgitation.

An intensified pulmonary second sound requires a strong right ventricle and an intact tricuspid valve, and is not always present. In general terms, the area of diffusion of a mitral regurgitant murmur is toward the left of the apex-beat. Whatever may be its character, the murmur is generally loudest at its commencement. A loud systolic murmur at the apex, and not heard at the back, is probably not produced by mitral reflux.

As at the aortic orifice, so at the mitral, stenosis and regurgitation are apt to occur in the same individual, giving rise to a combined presystolic and systolic murmur, which is a continuous murmur that begins shortly after the second sound of the heart and often continues until the second sound commences. The two sounds, although mingling to form one murmur, can, in the majority of cases, be readily distinguished from each other, for the point of maximum intensity and the very limited area of diffusion of a presystolic murmur readily distinguish it from a mitral systolic which is audible in the left scapular region. It is important to recognize the existence of both these {674} murmurs in estimating the prognosis in any case. Guttman mentions a case where five distinct murmurs were combined and yet clearly distinguishable.

DIFFERENTIAL DIAGNOSIS.--It is usually not difficult to recognize mitral regurgitation. The seat and rhythm of the murmur and its area of diffusion are sufficient to distinguish it from other cardiac murmurs. The character of the pulse, the symptoms referable to the right heart, and the pulmonary complications will also assist in its diagnosis.

It may, however, be mistaken for aortic obstruction, since both give rise to a systolic murmur, for tricuspid regurgitation, for fibroid disease of the heart, and for roughening of the ventricular surface of the mitral valve or of the ventricular wall near the aortic orifice.

The diagnosis between mitral regurgitation and aortic stenosis has already been given (see page 657).

Mitral and tricuspid insufficiency both produce a systolic murmur, but a mitral regurgitant murmur has its maximum of intensity at the apex, and is conveyed toward the left axillary and scapular regions, while the maximum intensity of a tricuspid regurgitant murmur is to the right of the base of the xiphoid cartilage, and it is transmitted upward and to the right: the area of transmission establishes the diagnosis.

Pulmonary symptoms are prominent in mitral reflux, and absent in tricuspid regurgitation. The pulmonary second sound is markedly enfeebled in tricuspid regurgitation, and markedly intensified in mitral regurgitation.

Fibroid disease of the heart may produce a systolic apex murmur, but it is an exceedingly rare disease, a pathological curiosity.[20]

[Footnote 20: In the Pathological Transactions (1874, vol. xxv. p. 64) Fagge records a few cases, and mentions that perhaps one positive indication of fibroid disease of the heart, rather than of a valvular lesion, may be found in its resisting treatment with greater obstinacy.]

Roughening of the ventricular wall gives rise to a murmur which has its maximum intensity at the base of the heart, and is transmitted along the aortic arch and into the vessels which spring from it in the thorax.

The vibration of an irregular chordæ tendineæ stretched across the aortic orifice, its extremities being inserted into opposite walls of the ventricle, may produce a systolic musical murmur, but the line of its transmission will correspond to that of an aortic obstruction. A systolic mitral murmur due to the sudden rupture of one or a number of the valve-flaps, of the papillary muscles or tendons, is accompanied by a loud systolic blowing murmur, which is immediately accompanied by all the urgent symptoms of acute pulmonary congestion.

Pulmonary Obstruction.

On account of the infrequency of disease of the pulmonic valves very little is known of the phenomena to which such diseases may give rise. In fact, they are so rare that there is no written history of their subjective symptoms; their diagnosis is only arrived at by exclusion, and they cannot be recognized except by the physical signs which attend them.

As has been already stated, endocarditis in the right heart is rare, except in intra-uterine life, and the various conditions of the aorta, atheroma, aortitis, etc., which I have mentioned in the etiology of aortic valvular disease have no analogues in the pulmonary vessels.

Usually, valvular disease of the right heart is the sequela of lesions in the left. It must be remembered, however, that the pulmonary artery may become atheromatous. I have already shown (see p. 666) how certain valvular diseases of the left heart may induce such a pathological condition. But even under such conditions disease of the pulmonary valves is rare. Balfour {675} believes that constriction of the pulmonary artery may occur at various periods of intra-uterine life. As a rule, the pulmonary valves are subject to no lesions except congenital malformation.

MORBID ANATOMY.--Bertin records an instance of pulmonary obstruction where the valves, distorted and adherent, formed a horizontal septum across the orifice, it being barely one-fourth of an inch.

A rigid tricuspid valve has been found to be the cause of obstruction at the pulmonary orifice, the pulmonary valves themselves being normal. A few autopsies have revealed obstructions at the pulmonary artery, caused not so much by valvular defect as by aneurisms, tumors of the pericardium or of the anterior mediastinum, enlarged bronchial glands, or pressure of a solidified lung.

The pulmonary artery may be occluded just beyond the valves by a cancerous tumor, and there are examples where a phthisical process in the left lung has induced it.

A murmur indicative of pulmonary obstruction may be produced by a cardiac thrombosis.

I have placed these statements under the head of its morbid anatomy for the reason that they cannot be appreciated and their pathological significance realized during life.

Reasoning from analogy, obstruction at the pulmonary orifice ought to be followed by compensatory hypertrophy of the right ventricle and accompanied by tricuspid regurgitation and dilatation of the right auricle.

Ormerod records 3 cases[21] where pulmonary obstruction was diagnosticated during life, and where the post-mortem proved the accuracy of the diagnosis: 2 of these cases occurred in men under twenty-eight, and the other in a woman of twenty-one. In 2 of these cases all the other cardiac valves were healthy. The pulmonic orifice would barely admit the introduction of a goosequill. Warburton Bigbie mentions a case (man æt. eighteen) where reflux and stenosis at the pulmonary orifice coexisted. There were four valves, and these were incompetent. All the other valves were normal.

[Footnote 21: Edin. Med. and Surg. Journ.]

Congenital stenosis of the infundibulum of the right ventricle is the probable result of foetal myocarditis or of syphilis.

I have never met but two pulmonic obstructive murmurs where subsequent autopsies were obtained. In both cases it was found that the murmur had been produced by mediastinal tumors pressing on the pulmonic artery so as to diminish the calibre.

ETIOLOGY.--Pulmonary stenosis is rarely the result of endocarditis or of degenerative changes in the pulmonary artery. Bertin states that when abnormal communication between the two sides of the heart has existed, the arterial blood has excited endocarditis in the right heart.

Syphilis has been advanced as a possible cause of degenerations at the pulmonic orifice.

SYMPTOMS.--The only rational symptoms that have been noted in the few recorded cases of pulmonic disease admit of manifold explanations, and no one is either constant or diagnostic. In some cases anæmia existed, in others there were cardiac palpitation, dyspnoea, cyanosis, and dropsy; but none of these belong exclusively to a pulmonic lesion nor do they necessarily depend upon it.

Physical Signs.--Inspection, palpation, and percussion give negative rather than positive results. In a few instances palpation may give a systolic thrill confined to the second left intercostal articulation. Such a frémissement results both from roughness and contraction of the pulmonic orifice.

Auscultation.--A systolic murmur is heard with its maximum intensity directly over the pulmonic valves; it is very superficial, and consequently {676} very distinct, and it is limited in its diffusion. It is never heard at the xiphoid cartilage nor along the course of the aorta. If it has an area of diffusion, it is toward the left shoulder. The murmur is loud and soft in character, sometimes bellows. It is not audible in the vessels of the neck nor is it attended by arterial pulsation.

When phthisical consolidation partially occludes the pulmonary artery, a loud but soft systolic murmur is heard, which is sometimes high-pitched and musical, and often entirely suspended during a full inspiration. In some few instances there is a bruit de diable in the jugular veins.

DIFFERENTIAL DIAGNOSIS.--It is possible to confound a pulmonic obstructive murmur with a mitral regurgitation which is propagated upward into the left auricular appendix. But the area of a mitral regurgitant is also backward, and by this it could be distinguished from a pulmonic obstruction. Besides, in mitral disease the pulse is very different from the pulse of pulmonary stenosis.

Aortic stenosis can hardly be mistaken for pulmonary obstruction, for the arterial pulsation, the peculiar pulse, and the transmission of the murmur into the arteries of the neck will suffice to discriminate between them.

An aneurism at the sinus of Valsalva may produce a systolic pulmonary murmur by the pressure which it produces upon the pulmonary artery. It would be impossible to distinguish it from a pulmonic stenosis.

The diagnosis of pulmonary obstruction is usually reached only by exclusion.

Pulmonary Regurgitation.

This form of valvular lesion is exceedingly rare; indeed, many doubt its occurrence. The lesion seldom occurs except as the result of injury or congenital defect, and there are but few well-authenticated cases in medical literature.[22]

[Footnote 22: Path. Trans., vol. xvi. p. 74.]

The statement[23] that the pulmonary valves exhibit a cribriform condition nearly as often as the aortic is not sustained in this country by the results of post-mortems. In one of the cases to which I have referred (p. 675) as an example of pulmonary stenosis the valves were likewise found insufficient. In Bigbie's case (referred to on p. 675), where there were four flaps to the valve (producing obstruction), there was marked insufficiency coexisting.

[Footnote 23: Dis. of the Heart, Bellingham.]

The morbid anatomy, etiology, and rational symptoms do not require a separate consideration. The anatomical appearances are the same as those found in similar conditions of the aortic valves, and the etiology and rational symptoms are the same as those of pulmonic stenosis.

Physical Signs.--Theoretically, pulmonic regurgitation should be accompanied by a diastolic murmur having its maximum intensity over the pulmonic valves, and its area of diffusion should be downward and toward the xiphoid cartilage. It should be soft and blowing in character. This murmur is rarely heard alone: it is usually associated with obstruction at the same orifice or with some murmur whose origin is on the left side of the heart.

Niemeyer states that dyspnoea, hemorrhagic infarction, and consumption of the lungs have followed insufficiency at the pulmonary orifice. No other authority mentions any such symptoms, while the assignment of valvular disease as a cause of phthisis is not based upon clinical facts.

With a pulmonic regurgitant murmur there should be on palpation and percussion physical evidences of hypertrophy and dilatation of the right heart, the rationale of whose production would be identical with that which was considered in aortic regurgitation. I have never heard a regurgitant pulmonic murmur.

{677} DIFFERENTIAL DIAGNOSIS.--The murmur of pulmonary regurgitation may be mistaken for that of aortic regurgitation. The points in connection with their differentiation are fully discussed on p. 664.

The PROGNOSIS and TREATMENT are identical with those of the former lesion.

Tricuspid Stenosis.

This valvular lesion is so rare that there are no established rules for its diagnosis.

Its MORBID APPEARANCES and ETIOLOGY are the same as those of pulmonic stenosis.

The SYMPTOMS of tricuspid stenosis would be those due to obstruction to the entire venous circulation. The right auricle would be dilated, and there would be visceral enlargements in the abdomen, cyanosis of the face and extremities, scanty and albuminous urine, hemorrhoidal tumors, headache, dizziness and vertigo due to passive cerebral hyperæmia, and finally general anasarca. The few recorded cases were associated with mitral stenosis with one exception, a case of Bertin's.[24]

[Footnote 24: Traité des Maladies du Coeur, Obs. 17.]

In a case exhibited by Quain the tricuspid flaps, thick and opaque, were united for one-third of their extent. In the other cases the valve-flaps formed a diaphragm whose central opening varied in size, admitting only the point of one finger. In every condition of tricuspid stenosis the heart was enlarged.

Tricuspid stenosis (as in pulmonic stenosis) may be the result of pressure of tumors.

In all well-authenticated cases the chief symptoms seem to be extreme lividity, palpitation, and dyspnoea.

Physical Signs.--Inspection reveals general cyanosis. The jugulars are turgescent and exhibit presystolic pulsation. This pulsation is sometimes the only inconvenience the patient suffers.

Palpation may discover a venous thrill at the base of the neck.

Percussion may show the right auricle to be greatly enlarged, and cardiac dulness will be increased laterally and toward the right.

Auscultation.--Tricuspid stenosis should be attended by a presystolic murmur whose maximum intensity would be at the lower portion of the sternum just above the xiphoid cartilage. This murmur may be propagated faintly toward the base, but never toward the apex of the heart. It is sometimes accompanied by fremitus.

Hayden offers the following diagnostic point: The murmur of mitral stenosis (without which tricuspid stenosis never occurs) is limited to the apex region; a murmur of the same rhythm is produced at the sternum by tricuspid stenosis, "and between these two localities there is a point where no murmur can be heard."

It is unnecessary to consider its differential diagnosis.

The lesion would be diagnosticated (if at all) by exclusion, and prognosis and treatment would depend on the gravity and sequelæ of the accompanying condition--viz. Mitral Stenosis (q. v.), for the rule is, that stenosis of the tricuspid never occurs unless there is extensive mitral obstruction, and the latter condition is always the predominant one.

Tricuspid Regurgitation.

Regurgitation at the tricuspid orifice is generally secondary to mitral stenosis or regurgitation; primary disease of the tricuspid valves, however, is not infrequent.

{678} MORBID ANATOMY.--The valvular lesions which lead to tricuspid insufficiency are similar to those which produce mitral insufficiency. The valves are thickened, shrunken, and opaque, the papillary muscles are shortened, thickened, and the chordæ tendineæ undergo similar changes and are sometimes adherent.

The valves or the chordæ tendineæ and columnæ carneæ may rupture; in either case acute and extensive insufficiency results, as has been stated. Acute endocarditis of the right heart is rare in adult life, but when it occurs the tricuspid valves are its primary and principal seat.

The reason for this is found in their anatomical structure and in the tension to which they are subject in diseases of the mitral valves. They are rarely the seat of rheumatic endocarditis or calcareous degenerations.

Ulcerative endocarditis is seldom met with in the right heart. In a case recorded by Charcot and Vulpian one of the tricuspid valves was softened and perforated, presenting numerous vegetations. Scattered abscesses in the lungs were found in this case.

Any infection through emboli from the tricuspid flaps will produce secondary effects within the thoracic cavity. The first effect of tricuspid regurgitation is dilatation of the right auricle; following this there will be more or less hypertrophy of its walls. As soon as the valves in the subclavian and jugular veins are no longer able to resist the regurgitant current jugular pulsation follows. But before this occurs the tributaries of the inferior cava and the organs to which they are distributed will become greatly engorged, for they have no valves to resist the regurgitant current, as are found in the veins coming from the upper part of the body. The inferior cava and the hepatic veins sometimes become enormously distended under these circumstances, and the liver will show the peculiar section that has gained for it the name of nutmeg liver.

Following the hepatic changes, the skin assumes a dingy yellow hue. When this is combined with cyanosis it produces a peculiar greenish tint which is only met with in heart disease. The spleen enlarges and hardens; the mucous membrane of the stomach is congested, ecchymotic, and often presents numerous hemorrhagic erosions. Intestinal catarrh is subsequently developed, and the general venous congestion within the abdominal cavity is exhibited by hemorrhoids and ascites. The kidneys become congested and stony, and thrombi may form in the femoral vein and induce subsequent pulmonary infarctions.

The stasis in the veins below the diaphragm is accompanied by transudation of serum--first in the ankles, and thence the dropsy progresses upward until the patient may finally reach a condition of general anasarca. The obstruction to the general systemic circulation which results may subject the left ventricle to so much extra labor that it hypertrophies, and then we have the infrequent occurrence of disease of the left heart following that of the right.

Since tricuspid reflux has mitral disease for its principal cause in abnormal cases, the heart becomes greatly enlarged and a condition of extreme cardiac dilatation and hypertrophy is reached.

ETIOLOGY.--As has been stated, the most frequent cause of tricuspid regurgitation is mitral disease, either stenosis or regurgitation. Any condition of the lungs which will produce hypertrophy and dilatation of the right ventricle will lead to it; it is met with in extensive pulmonary emphysema, in cirrhosis of the lung, and in extensive chronic bronchitis. Balfour regards chronic bronchitis as its most frequent cause after mitral stenosis.

It is possible for any valvular disease in the left heart, when of long duration, to lead to tricuspid regurgitation. From all these causes the rationale is {679} the same: the abnormal amount of blood in the right ventricle presses with undue force against a valve, which physiologists regard as normally slightly insufficient; the stress upon the valve-flaps and the valvular attachments is such that endocardial inflammation is excited at the part subject to the greatest strain, and valvular insufficiency is the result.

It is possible for disease of the tricuspid valves to result from any of the causes which have been enumerated on p. 666 as etiological factors in valvular diseases.

SYMPTOMS.--Tricuspid regurgitation being in the majority of cases secondary to some other valvular disease or some chronic pulmonary affection, its symptoms during the early stages are vague and masked by those of the primary disease. But as soon as the valves become so insufficient that the venous return is markedly impeded, a train of symptoms is developed which has its origin in the visceral derangements already referred to.

In addition to these symptoms there may be, with extensive tricuspid regurgitation, cardiac palpitation, cardiac dyspnoea, and marked irregularity in the force and rhythm of the heart. The liver and spleen are enlarged, the skin becomes dingy, and there is obstinate constipation with hemorrhoids. The liver is likewise rendered very liable under such circumstances to attacks of interstitial hepatitis. Venous stasis is evinced by dyspepsia, nausea, vomiting, and hæmatemesis. The secretion of the kidneys is scanty, dark-colored, of high specific gravity, often containing albumen and casts.

Passive cerebral hyperæmia is marked by headache, dizziness, vertigo, and muscæ volitantes, and there is a peculiar mental disturbance which is not met with in any other form of heart disease.

Late in the disease, if the patient is placed in a horizontal position, the face becomes turgid and blue, and if he remain long in the recumbent position stupor and coma may supervene. Jugular and epigastric pulsation are characteristic physical signs.

A very late symptom is dropsy, which begins at the ankles and extends upward until there is general anasarca. It is a point to be noticed that in the dropsy from tricuspid reflux the genital organs suffer slightly if at all.

Physical Signs.--Inspection.--In extensive tricuspid disease the area of the cardiac impulse is increased more than in any other valvular lesion. This area sometimes extends from the nipple to the xiphoid cartilage, and it may reach as high as the second right intercostal space. There is a visible impulse in the jugular veins, more apparent in the right than in the left. Sometimes the veins in the face, arms, and hands, or even the thyroid and mammary veins, are seen to pulsate.

Palpation.--The apex-beat is indistinct, except in cases where there is marked hypertrophy of the left ventricle. Pulsation occurs in the epigastrium, which may be due to reflux into the enlarged hepatic veins or to the fact that the dilated and hypertrophied right ventricle so presses on the liver that the impulse is conveyed through the diaphragm with each cardiac pulsation. Guttman thinks epigastric pulsation is due wholly to reflux into the veins of the liver, and not to right ventricular pulsation.

{680} Early in the disease the impulse in the jugulars is confined to the lower part of the vessels, particularly to the sinus. Beyond this point the vein merely undulates. Later, a systolic pulsation is felt as high up as the angle of the jaw, and may be accompanied by distinct though feeble presystolic pulsation.

The liver may first simply undergo systolic depression, chiefly at the left lobe; secondly, the whole liver may have an impulse coming from an enormously dilated vena cava; and thirdly, the systolic pulsation of the veins within the organ may give to it a palpable expanso-pulsatory movement. The hepatic pulsation is rhythmical with the cardiac impulse. In rare cases it precedes jugular pulsation. Sometimes pulsation is felt in the femoral veins.

Sphygmographic tracings of the jugular pulse show it to be dicrotic.

Percussion shows an increase in the area of cardiac dulness to the right and upward, sometimes as far as the second intercostal space.

Auscultation.--The murmur of tricuspid insufficiency is heard with, or takes the place of, the first sound of the heart; it is superficial, of low pitch, blowing, soft, and faint, and is heard with the greatest intensity over the lower part of the sternum, at its left border, between the fourth and sixth ribs. It is rarely audible above the third rib or to the left of the apex-beat. This murmur is transmitted from the region at the base of the xiphoid cartilage upward and to the right from one to two inches. Sometimes it is heard only over a very limited area, and then it may be overlooked.

DIFFERENTIAL DIAGNOSIS.--A tricuspid regurgitant murmur may be confounded with that due to aortic obstruction, pulmonic obstruction, and mitral regurgitation. A tricuspid regurgitant murmur is never audible above the third rib; is not accompanied by an accentuation of the second sound over the pulmonary artery, but by jugular and epigastric pulsation; and is heard with maximum intensity near the base of the ensiform cartilage. These points are sufficient to differentiate it from an aortic or pulmonary obstructive murmur. The differential diagnosis between it and a mitral regurgitant murmur has been given.

PROGNOSIS IN VALVULAR DISEASES OF THE HEART.--Any statements as to the duration of life in valvular diseases of the heart, and their relative frequency as a cause of death (especially of sudden death), must be based upon personal observation, and necessarily will differ with different observers.

In order to establish, if possible, a basis of comparison for the different valvular lesions, I give a résumé which I have made of 81 cases, in all of which autopsies were made and the diagnosis of valvular disease verified.[25]

[Footnote 25: Med. Rec. N.Y., April 1, 1870, p. 66 et seq.]

In 14 cases of various valvular lesions, each of which was accompanied by cardiac hypertrophy and dilatation, 50 per cent. of the deaths were due directly to the valvular lesion. In 1 of these, where there was stenosis at both auriculo-ventricular orifices, death was sudden.

In 15 cases of valvular disease, in which there was only cardiac hypertrophy, there were 11 deaths from the heart lesion. In 5 of these death occurred suddenly, and these 5 sudden deaths were all directly due to the heart lesion.

In 6 cases of valvular disease accompanied by dilatation alone, 4 deaths resulted directly from the heart lesion, and 2 of these were sudden.

In 15 cases where the aortic valves were involved (either calcified, rigid, or atheromatous) the heart lesion was not the cause of death in any case. Of these 15 cases, sudden death occurred but in 2; in 1 there were firm and long-standing pericardial adhesions, and in the other cerebral apoplexy.

In 12 cases of calcification of the mitral valve, no death occurred as the direct result of the valvular lesion, and there were only 2 sudden deaths, both from cerebral apoplexy.

{681} The aortic and mitral valves were diseased in 14 cases; in 2 of these only did death result from the heart lesion, and the only three sudden deaths in this class were from uræmia, apoplexy, and croupous laryngitis.

The aortic and pulmonic valves were both diseased in 3 cases which died suddenly, and in no instance was death due directly to the heart lesions.

In 2 cases there was disease at the aortic, mitral, and tricuspid orifices, and no sudden death.

Thus it will be seen that of these 81 cases, in 24 only was death due directly to the heart lesion. There were only 8 sudden deaths due directly to the heart lesion.

The results of personal, clinical, and pathological observation lead me to the opinion that the loudness, harshness, and the area of diffusion of any cardiac murmur have little to do with its prognosis.

I deduce from the above-mentioned cases that cardiac murmurs rarely necessitate a bad prognosis unless hypertrophy and dilatation coexist; but so soon as the signs of considerable dilatation and hypertrophy are present a great variety of complications are liable to occur.

In 1870, I had a patient sixty years of age with extensive aortic reflux, who had been under my observation eight years, during which time he had three attacks of pneumonia. There were no appreciable signs of cardiac dilatation in his case.

Walshe says: "The order of relative gravity, as estimated not only by their ultimate lethal tendency, but by the amount of complicated miseries they inflict, is--1, tricuspid regurgitation; 2, mitral obstruction and regurgitation; 3, aortic regurgitation; 4, pulmonic obstruction, 5, aortic obstruction."

The following are conditions which render the prognosis in each valvular lesion more or less unfavorable:

In aortic stenosis the prognosis is less grave than in any other valvular lesion. Life may be prolonged and good health enjoyed for many years. Yet it must be remembered that extensive aortic stenosis rarely exists without attendant regurgitation.

So long as the hypertrophy of the left ventricle compensates for the obstruction, the prognosis is good; but when the hypertrophied walls fail to overcome the obstruction, dilatation begins, and the ventricular systole becomes feeble and intermitting, and the arterial supply to the brain is so much diminished as to lead to cerebral anæmia.

If after sudden exertion or violent muscular effort there is interruption or great irregularity in the heart's action, sudden death may occur from a complete arrest of the ventricular systole.

Evidences of excessive hypertrophy and dilatation, the occurrence of syncope, signs of cerebral anæmia, attacks of vertigo, great muscular prostration, continued and marked paleness of the face, and irregularity of the pulse, render the prognosis exceedingly unfavorable in aortic stenosis.

If the presence of vegetations can be determined, there is danger from cerebral embolism.

When there are no evidences of alterations in the ventricular walls after an aortic obstructive murmur has existed for some time, it may be assumed that no vegetations exist on the valves, and that the murmur is not due to extensive aortic stenosis, and consequently is not dangerous to life.

When the mitral valves become involved, the combined lesions render the prognosis unfavorable.

Death may result from cerebral complications, pulmonary oedema, or cardiac degeneration.

Aortic insufficiency is a much graver form of valvular disease than aortic stenosis. It is difficult to estimate the probable duration of life in aortic {682} insufficiency, for it frequently gives rise to no symptom that would lead to its diagnosis until it is far advanced. Twenty-one days and five years are the extreme limits that have been recorded. It must always be borne in mind in estimating the factors for and against a good prognosis that in no other valvular lesion is sudden death so liable to occur. Yet the record of the cases which I have given (page 680) indicates that mitral stenosis is nearly, if not quite, as frequently a cause of sudden death.

A diseased valve can never be restored to its normal functions, and the shorter and more gushing the murmur the more extensive the regurgitation. The effects of the regurgitation must be carefully estimated before a prognosis can be given in any case. When one aortic flap is puckered and shrunken, the other two may elongate and compensate for the patency. But this occurs only in very young subjects.

Aortic regurgitation is, however, more serious in the very young than in adults. In children the valvular changes are less atrophic and more inflammatory in character.

Where the disease is met with in middle life, in those who daily undergo severe mental or bodily strain, the prognosis is unfavorable. And when in such patients there are the evidences of arterial degeneration or a tendency to it, the dangers are greatly increased, for the hypertrophied ventricle drives out the blood from its dilated cavity with greater than the normal force, and the vessels being weakened there is great danger of their rupture; hence the frequent occurrence of apoplexy and infarctions. In the very old I have seen aortic incompetence last a long time and cause little inconvenience.

Again, the prognosis is bad when cyanosis and dropsy result from the failure of a dilated and hypertrophied left ventricle to empty itself. This weakness is the result of that interference with the coronary circulation which brings about impaired nutrition, and therefore degeneration of the heart-walls.

When mitral insufficiency is secondarily induced, then obstruction to the systemic circulation leads to induration of the liver and kidneys, which interferes with the performance of their functions and hastens the fatal issue.

Sudden rupture of a valve or valvular disease that has developed very rapidly is more dangerous than when the valvular insufficiency is slowly developed. The flap or flaps involved can sometimes be determined during life, and then the prognosis will be more or less favorable according as the anterior or posterior are incompetent. In all cases the prognosis depends more upon the condition of the heart-walls and on the general nutrition than upon any other element.

When aortic regurgitation is complicated by aortic stenosis, mitral regurgitation, or by the vascular and visceral conditions resulting from the derangement of the circulation, the prognosis is exceedingly unfavorable. Death may result from embolism, apoplexy, dropsy, pulmonary oedema, from sudden cardiac insufficiency, or from visceral complications. When the radial impulse is felt a little after the apex-beat, it is always important to determine whether the action of the heart remains regular under mental excitement or violent physical exertion: if it does, the prognosis is far better than when it becomes irregular.

Mitral stenosis admits of but slight compensation; if extensive, it is always a grave disease. The prognosis in any case can be estimated by the severity of the thoracic symptoms. When physical exertion greatly exacerbates the thoracic symptoms, the prognosis is especially bad; for during violent exercise such patients are not only liable to pulmonary congestion and oedema, but to pulmonary infarctions and pulmonary apoplexy with large extravasations.

Where mitral stenosis is extensive it ranks next to aortic regurgitation in its danger of sudden death. The statistics furnished by Bellevue Hospital show sudden death to occur as often in mitral stenosis as in aortic reflux.

{683} Congenital mitral stenosis is not dangerous, and does not cause much embarrassment, for it is invariably associated with hyperplasia of the pulmonary arterial system. The later in life mitral stenosis occurs, the more unfavorable the prognosis.

Mitral regurgitation uncomplicated by any other valvular lesion gives rise to very little disturbance of the systemic or capillary circulation. It is more often fully compensated for than any other valvular lesion. The changes which lead to it are of slow growth and their tendency is to remain stationary. Patients with a moderate regurgitation at the mitral orifice suffer very little except during or after violent physical exercise, and, were it not for the slight dizziness which attends it, it would pass unnoticed. As long as the compensatory hypertrophy of the right ventricle is sufficient to overcome the obstruction to the pulmonary circulation, patients with this form of heart disease may not suffer from dyspnoea even after violent physical exercise. As regards the duration of life, the prognosis in mitral regurgitation is good. When, however, mitral stenosis and regurgitation coexist, the liability to sudden pulmonary complications becomes so great that a very guarded prognosis must be given; and it must be remembered that combined reflux and stenosis at the mitral orifice is a frequent combination.

In very many instances it is unnecessary to tell a patient with mitral reflux that he has an incurable heart disease, for with no other valvular lesion the individual may live to advanced life. But when it is combined with mitral stenosis it must be regarded as a very serious form of valvular lesion. As soon as symptoms occur that show failure of the right heart, the prognosis becomes unfavorable. Oedema of the extremities or fluid in any of the serous cavities, cyanosis, dyspnoea, and hæmoptysis, are indications of such failure.

Death may result from general anasarca, from serous effusions into the pleuræ, peritoneum, or pericardium, from pulmonary oedema and congestion, or from heart-insufficiency.

Extensive obstruction or regurgitation at the pulmonic orifice would necessarily lead to serious results, but there are no reliable data upon which the prognosis can be based.

The prognosis in tricuspid obstruction and regurgitation, when associated with mitral disease, is very grave; but it is not as bad as when it results from chronic bronchitis and pulmonary emphysema.

When in any case jugular and epigastric pulsation are marked, the changes in the various organs of the body already referred to rapidly ensue. Walshe says that "tricuspid regurgitation is the worst of all valvular lesions." Patients with tricuspid reflux are in extreme danger from intercurrent attacks of acute pulmonary hyperæmia.

Tricuspid disease, of all valvular lesions, leads most rapidly to cyanosis and dropsy.

TREATMENT.--The treatment of aortic stenosis and of aortic regurgitation may be summed up under three heads--viz. rest, diet, and regimen.

Rest is most important; it must be mental as well as physical; the appetite, emotions, and passions must be kept under perfect control: these indications are best maintained by a sedentary country life. Straining, especially when the hands are above the head, should be carefully avoided.

The stomach also must have all the rest compatible with the most perfect nutrition; it is frequently a difficult matter to combine both indications, for it should be remembered that the more perfectly the nutritive processes are maintained the longer will the cardiac muscle resist degeneration. Sugar, sweet vegetables, and animal fat must be sparingly indulged in. The food should consist of nitrogenous, albuminoid material, and should be taken in quantities that do not disturb the heart's action.

{684} In aortic incompetence patients in sleeping should assume, as nearly as possible, a horizontal posture. By lying on their backs they lower the height of the distending column of blood, and thus relieve both the cardiac circulation and the tendency to pulmonary congestion. Sometimes, when defective aortic pressure reacts injuriously on both the gastric and hepatic secretions and limits both their supply and their efficiency, moderate alcoholic stimulation may be cautiously employed to tide over a weakly period. The bowels should be gently moved once daily. That the cutaneous circulation may be active the body should be warmly clothed. Any prolonged exposure of the surface to cold is to be avoided. In winter the warm bath may be occasionally used, and in summer the patient is frequently benefited by a warm sea-water bath.

Medicinal agents are not to be resorted to until the cardiac hypertrophy fails to be compensatory. Then relief is demanded for the failing heart-power. In aortic regurgitation with feeble heart-action the tincture of digitalis and the tincture of the perchloride of iron are to be given in ten-minim doses three times a day. The iron is especially indicated whenever anæmia is evidenced. Digitalis is given to produce a sedative action, and therefore should be given in very small doses and regulated according to its effects on each patient. An infusion of the English leaves is the preparation which is most reliable, although the tincture, if fresh and well prepared, is equally good. When rapid and immediate action is demanded, digitalis may be given hypodermically. There is one guide to its use not unimportant to remember: that is, as long as it causes an increase in the flow of the urine it is safe to continue its use. When vertigo and syncope are prominent symptoms quinine and strychnia may be given with the digitalis. When the heart in aortic reflux acts with violence and rapidity, and the arteries are in a state of high tension, aconite will be found of service in quieting the heart's action. In aortic incompetence small doses of arsenic seem to have a stimulating effect, especially when given with digitalis and iron. Iron may disturb the stomach, arsenic seldom if ever does. It is always a safe rule when giving iron to administer at the same time a bitter vegetable infusion, as quassia or columba.

When the hepatic and gastric vessels are engorged, three or four leeches over the liver or epigastrium, followed by a warm fomentation, will afford temporary relief.

At no time should a large quantity of fluid be taken into the stomach. Symptoms of angina pectoris, with local pain and dyspnoea, are evidences of aortitis. This demands the application of leeches over the sternum and continued small doses of mercury.

The treatment of dyspnoea, dropsy, pulmonary oedema, and other late and distressing symptoms will be considered in connection with mitral disease. Sometimes the pain of aortic disease is so severe as to require an anodyne for its relief: opium must not be given by the mouth, but the sulphate or the hydrochlorate of morphine can be safely given hypodermically. The severe angina-like pain of aortic regurgitation can often be promptly relieved by the nitrate of amyl.

Barlowe and Fagge both advise senega and ammonia carbonate for the less severe effects of aortic reflux. They advance no reason for the use of these drugs, but their cases show that they have a markedly beneficial effect. All authorities unite in regarding aortic insufficiency as less amenable to treatment than other valvular lesions.

In all cases the idiosyncrasy of each patient should be carefully considered.

No treatment can restore a diseased valve to its normal condition, or prevent, for any considerable time, cardiac dilatation and hypertrophy when the normal function of the valves is greatly interfered with.

{685} The first step in the treatment of a serious lesion at the mitral valves is to make the patient clearly understand his exact condition, that he may see the reasonableness of the advice given, for his treatment for the most part must be carried on by himself. A patient must be fully persuaded of its necessity before he will regulate his habits and mode of life in accordance with the requirements of his case. The rules as to nutrition are the same as those to be observed in aortic stenosis and reflux. There should be a gentle and regular daily evacuation from the bowels. Straining at stool must be avoided, and any use of alcohol, strong tea, coffee, and tobacco is to be prohibited. If in either form of mitral valvular disease the patient is anæmic, iron should be given. This is given as a food to such patients, and is best administered about half an hour after meal-time. Ten or twenty grains of Vallette's mass may be given with benefit to anæmic patients two or three times a day for a long period.

Patients with mitral reflux should avoid a prolonged use of the voice, especially in speaking or singing. Small doses of quinine and strychnine, alternating with the administration of iron, are often of service. If there is anorexia, infusion of quassia or columba may be given with the iron. The triple phosphates of iron, quinine, and strychnine, or small doses of dilute sulphuric acid, will be found to improve the condition of these patients when they show signs of extreme debility.

In every case of mitral disease there comes a period when the pulmonary hyperæmia shows that the compensation of the right heart has failed. An adjustment of the heart to the circulation is now effected by the judicious administration of digitalis. Digitalis should only be given at those times when the heart-failure is imminent and there is marked pulmonary congestion. Half an ounce of the infusion every two hours for twenty-four or forty-eight hours is often required to overcome the heart-failure. The time will come when digitalis ceases to have its sustaining effect upon the heart-muscle; hence it should always be most sparingly and carefully used, and the patient should never be allowed to use it continually.

When the pulse is rapid, feeble, and irregular, more time is needed for the flow of blood into the ventricle, and greater force and regularity in the ejection of the blood from that ventricle are demanded. Digitalis fulfils all these conditions: the pulse becomes regular, beating about sixty per minute, full and forceful. The urine, before scanty, now becomes abundant and normal. Pulmonary engorgement diminishes, and commencing dropsy gradually but totally disappears.

Hayden advises ten minims of the spirits of chloroform and fifteen minims each of the tincture of digitalis and the tincture of the perchloride of iron in an ounce of water every three hours.

Whenever asystolism is present or suppression of urine is threatened, digitalis should be given whether the other indications are present or not. In most cases of mitral stenosis it is best to avoid the use of digitalis as far as possible.

The dropsy which accompanies advanced mitral regurgitation may be promptly relieved by compound jalap powder, combined with calomel in sufficient quantity to produce prompt and free catharsis. In some cases of cardiac dropsy, squill, juniper, brown cream of tartar, and copaiba act as diuretics. This latter drug is best exhibited in the form of the resin.

In mitral reflux a combination of digitalis and nitrous ether will often be found to act as a diuretic. In all cases when a diuretic is given in heart disease the loins should be cupped or warm poultices applied and the bowels freely purged. In copious hæmoptysis in cardiac disease ergotin may be given in full doses either by the mouth or hypodermically.

The hæmoptysis which accompanies pulmonary apoplexy of heart disease {686} sometimes temporarily relieves the dyspnoea. On this basis Dickenson and Fagge and other English writers recommend venesection for the relief of the pulmonary engorgement or heart-failure. Pain in the præcordial region which accompanies valvular insufficiency may sometimes be relieved by the application of leeches over the præcordial space. Hyoscyamus, hydrochlorate of morphia, nitrate of amyl, chloroform, and a belladonna plaster over the præcordial space have all been employed for the same purpose.

It is to be remembered that such pain is the cry of the heart-muscle for a higher degree of nutrition.

Bleeding in heart disease favors dropsy by thinning the blood and by diminishing the heart-power. It should never be resorted to except in great emergencies. Niemeyer advises arsenic and antimony in mitral valvular disease, but does not say in what cases or for what reason they are to be used. When in the late stages of mitral disease the free use of digitalis fails to regulate the pulse and to relieve the pulmonary engorgement, its prolonged administration does harm rather than good; but in every case of mitral disease where the drug has not been used it may be safely affirmed that its administration will give prompt relief.

If it becomes necessary to use an anodyne or hypnotic at any period in the course of mitral valvular disease, morphia hypodermically is to be preferred to all others.

The rules in regard to hygiene, diet, and exercise which have been given for the management of mitral disease are equally indicated in the management of pulmonary obstruction or regurgitation. Beyond this their treatment is purely symptomatic.

The treatment of tricuspid obstruction depends upon the gravity and sequelæ of the accompanying disease--viz. mitral. Stenosis of the tricuspid orifice never occurs until mitral obstruction is excessive, and the latter condition is always the predominant one.

The same rules of hygiene and diet which have already been given for mitral disease must be followed with the utmost care by those suffering from tricuspid reflux. The patient must lead a life of perfect quiet, and should live in a warm, equable climate. When occurring with mitral disease digitalis should not be omitted; for although the drug, by increasing the action of the heart, would seem to be injurious, yet it promotes ventricular contraction, and thus tends to relieve the tricuspid pressure. In tricuspid insufficiency with pulmonary emphysema this drug should be very cautiously exhibited, and its use or omission must depend upon the effects produced in each case. If the cerebral symptoms are exaggerated, it must be discontinued. The indications for the use of tonics, such as iron, quinine, strychnine, are the same and follow the same demands as in mitral disease. When venous engorgement demands prompt relief, drastic cathartics or the abstraction of a few ounces of blood from the arm will temporarily diminish the high venous tension. The treatment of the dropsy and the local oedema is the same as for similar condition occurring in mitral disease. There are many subsidiary remedies which will have to be employed for the relief of gastric, hepatic, and intestinal symptoms, which are often the most troublesome occurrences of this disease.

{687}

CYANOSIS AND CONGENITAL ANOMALIES OF THE HEART AND GREAT VESSELS.

BY MORRIS LONGSTRETH, M.D.

The questions involved in the subject of the congenital defects of the heart and its great vessels and their causes are not easy of settlement. In the first place, the seat, the extent, and the consequences of the deficiency or defect are not regular or constant. Secondly, the causes and the mode and date of their origin are involved in great obscurity. Their classification either on a purely topographical or on a purely etiological basis is almost impossible on the one hand, because the changes are so irregular and varying, and, on the other hand, because our knowledge of the primary cause or causes of the alterations is quite defective. The views which at the present time find most favor arrange the various malformations into classes according to the period of development of the foetus at which the arrest or change of tissue occurred--as it were, a chronological classification. The ideas in respect to the pathology or the pathological causes of malformed hearts have undergone great changes--changing in some degree pari passu with the mode of classification, and in great degree inducing and compelling such changes.

In early times deformed hearts were looked upon as monsters, curiosities, lusus naturæ. When a knowledge of foetal development and circulation was acquired the deformed heart was compared with the heart-formation in classes of a lower grade than mammals. Such were the beliefs of comparative anatomy and physiology that it was held that the human foetus was matured by stages from the forms found in the lowest invertebrates through the various ascending scales of the animal kingdom. This classification was, on the basis of comparative anatomy, purely anatomical. The underlying thought of such pathological teaching was that in the original ovum something was left out--an actual deficiency of parts which, when developed in the natural manner, made man different from the lower animals; or else, supposing these parts to have been originally present, there was a defect of plasticity, causing a failure of the proper adhesion of symmetrical portions. Excessive development was looked upon as a surplus of parts in the ovum, and by their growth certain of the openings of the heart were prematurely closed. In this view of the pathological alterations no expression of opinion was made how the excess or deficiency of structure was occasioned: the malformation was merely a failure of the parts to rise and pass through the various grades of development--a too rapid or a too slow growth of one or more of the various parts of the foetal heart. There was no reason assigned why the human ovum had in it deficiencies or excesses of material, and thus came to resemble in one of its parts the conditions found in lower animals.

About 1850, Dittrich of Erlangen, by his studies of inflammation of the heart during intra-uterine life, quite diverted public opinion from the older views of the subject. Peacock's earlier studies preceded this work by a few {688} years, and a few years later came Meyer,[1] who greatly extended the scope and influence of the inflammatory theory of Dittrich. Ten years later commenced the clinical recognition of congenital heart defects, and especially the anatomical changes in congenital narrowing of the pulmonary artery, by Von Dusch and by Mannkopff,[2] and by Stoelker.[3] Friedberg had, however, as early as 1844, published his studies of the stages of development of the circulatory organs in the human embryo, and had in accordance therewith divided the malformation of the heart into three groups, corresponding to the three periods of the heart's growth. This was the classification adopted quite independently by Peacock of London in his first publication in 1857. It was not until after Dittrich's studies[4] and Meyer's that any distinctive cause was assigned for the failure to develop.

[Footnote 1: Virch. Arch., Bd. xii., 1857.]

[Footnote 2: Ann. des Charité-Krankenh. zu Berl., 1863.]

[Footnote 3: Diss., Bern, 1865.]

[Footnote 4: See Dorsch's (his student) dissertation, Die Herzmuskelentzundung als Ursache angeborner Herzcyanose, Erlangen, 1855.]

Carl Heine,[5] and also Halbertsma, proposed a classification based on the quantitative and qualitative differences. Under the first division the former placed such changes as absence of the heart, deficiency of individual parts, abnormal smallness, atresia, and fissures; and, in the other direction, duplication of the heart as a whole or in its individual parts, and abnormal largeness. The qualitative differences were deviations of form, of position, and of the arrangement of the great vessels.

[Footnote 5: Angeborene Atresie d. Ostium arteriosum dextrum, Beitrag z. Lehre v. d. angeborenen Herzanomalium, Tübingen, 1861.]

Peacock's classification in his earlier edition (1858) was partly on the basis of the time at which arrest of development occurs, and partly on the degree of impediment to the circulation and the functions of the heart. In his second edition he adheres to the same classes, with slight modifications, thus: 1. Arrest of development early in foetal life (fourth to sixth week; heart with two or three cavities; single or imperfectly divided arterial trunk); 2. Arrests at a later period (sixth to twelfth week; imperfect auricular or ventricular septa; imperfect or misplaced vessels); 3. Those after the third foetal month (closure and patency of foetal passages; irregularities of valves, cavities, etc.).

Kussmaul (1865) published a very important work on malformations due to defects of the pulmonary artery,[6] and these malformations he considers under two general groups--viz. those having their origin before the ventricular septum closes, and those occurring after this period. His most valuable contribution to the subject is the importance which attaches to the distinction between primary and secondary defects or arrests of development--i.e. between an original alteration of growth or morbid condition, and those which follow from it as a necessary consequence. Of his classification, and of the importance of pulmonary artery malformations, a further description will be given.

[Footnote 6: Ueber angeborene Enge und Verschluss der Lungenarterienbahn, Freiburg, i. B.]

For study, one would wish to arrange the malformations in classes convenient for clinical purposes. For example, separate them into groups of the defects compatible with extra-uterine existence and those incompatible with adult life. Unfortunately, this division is not possible. We find many cases of defects involving originally the same seat: in one the individual lives many years, in another the obstruction immediately induces symptoms, and death soon comes. A classification according to the seat of the disease alone, if it could be made, would give the subject a simplicity equal to that of valvular heart disease in the adult. Here, however, we find such variations in the details of the alteration that if this principle of classification alone is {689} employed the confusion becomes very great. It would seem, therefore, that the principle first made use of by Kussmaul, of classifying the defects by distinguishing the primary malformations from their secondary effects, renders the subject the most simple, and at the same time affords the advantage of more readily understanding the mechanism of their production.

It will be useful to pass over seriatim, following the course of the foetal circulation, the various valves, orifices, and foetal openings to be able to comprehend which are most liable to defects or to see which defects most frequently occur, and also to find which alterations produce the greatest disturbance of the circulation.

1. The Foramen Ovale and Septum of the Auricles.--In markedly deformed hearts the entire septum may be in greater or less degree wanting, as seen in cases of the bilocular or trilocular organ. This defect is comparatively rare, and the foetus has but a short extra-uterine life. In other cases the septum is complete, but the foramen may be unusually large, and remain unclosed wholly or in part; perforations may be present, or the valve may merely fail to adhere. Of the latter cases, the patent foramen is found in conjunction with defects at other parts, while small sieve-like perforations or the mere non-adherence of the membrane--both of very common occurrence--may be owing to a temporary obstruction during the early hours of life or to any unknown cause, or may possibly be due to a reopening of the foramen from an acquired disturbance of the circulation. Opinions vary as to the mechanism of the closure of the foramen. Some consider it a passive process due to increased blood-pressure in the left auricle, coming from the entrance of the current of aërated blood from the lungs; others speak of it as an active process resulting from the excitation to contraction of the muscular fibres in the membranous valve. Whatever may be the mechanism, patency of the foramen ovale of undoubted foetal origin (excepting the minute perforations and oblique slits) must be looked upon in nearly every case as a secondary defect--secondary to an obstruction to the outflow of blood from the ventricles through the great arterial trunks, or it may be from the auricle itself through defect of the auriculo-ventricular orifice. In a vastly preponderating number of cases it results from pulmonary artery obstruction. The foramen may close, however, in such a case if an outlet is provided by the aorta through an open septum ventriculorum, or when this vessel arises from both ventricles. Narrowing or closure of the right auriculo-ventricular orifice, as a primary cause, can prevent the closure of the foramen ovale; primary narrowing of the tricuspid orifice is very rare, single or combined with other defects. In these cases the direction of the blood is from the right auricle to the left. There are, however, cases on record of patency of the foramen ovale in which the blood-current is from the left to right side, the reverse of the foetal course. Here the cause to be looked to is a congenital deficiency of the mitral orifice, or a narrowing, closure, or malposition of the aorta.

2. The Right Auriculo-ventricular Orifice and Tricuspid Valve.--A primary deficiency of this orifice and the valve guarding it very rarely occurs as a primary defect and uncombined with malformation of other parts of the pulmonary circulation. It does come in certain cases in conjunction with great narrowing of the pulmonary orifice or artery, but by no means commonly. If the pulmonary outlet is normal and in the usual position, the right auriculo-ventricular orifice is never found closed, although the leaflets have been seen defective, permitting regurgitation. In certain other cases the orifice and valve, as well as entire right ventricle, show a failure to develop, and all these parts appear shrivelled. This condition is a secondary result, due to a great deficiency of the pulmonary artery and narrowing of the pulmonary conus. The malformation of the pulmonary artery in such cases results from an unequal division of the truncus communis--the narrowing {690} of the conus generally from endo-myocarditis. The aorta is unusually large in diameter. The blood from the right auricle passes through the foramen ovale to the left side of the heart; the ductus arteriosus Botalli remains open, or in very rare cases the mixed venous blood reaches the lung through collateral channels. In rare cases the blood, in addition to the open foramen ovale, has a direct passage from the right auricle into the left ventricle.

3. The Pulmonary Artery and the Right Conus Arteriosus.--This situation presents by far the largest number of cases of congenital heart malformation of primary occurrence. The defects at this part require different interpretations according as they are found within the right ventricle or in the pulmonary artery itself. So frequent are the defects at these seats that Kussmaul bases his classification, for a large proportion of cases, on the malformation of the pulmonary artery track, and describes them as combined with defects secondarily resulting in other parts.

The narrowing or closure may exist either at the limit between the sinus and the conus of the right ventricle, the conus arteriosus may be uniformly narrowed, or the defective development may involve the orifice only or the whole length of the pulmonary artery. Many of these defects, resulting in closure or narrowing, are due, as Rokitansky was the first to show, to inflammatory changes. It is Kussmaul's great merit to have pointed to the fact that a very large proportion of all malformations owe their origin primarily to diseased conditions originating at this seat. The varieties of these defects and their secondary consequents will be described later.

4. Patency of the Septum Ventriculorum.--The degree of deficiency of the septum varies greatly. The entire partition between the ventricles may be wanting or exist in merely a rudimentary condition. Ordinarily, there is found a triangular, rounded, or oval opening in the septum close to the base of the heart, at the portion which in the normal heart consists of only a membrane (pars membranacea). Besides this usual opening, one, or even two, others may present themselves at other points of the septum, thus forming multiple communications between the cavities. In narrowing or closure of the pulmonary passage the septum is more or less deflected toward the left ventricle to allow a freer passage of blood from the right side of the heart through the open septum into the aorta. In other cases the passage of blood may be from the left ventricle into the right--the reverse of the usual direction. The defects of the septum are usually of a secondary character, dependent on primary malformation of other parts, and, as already said, chiefly those of the pulmonary track. They are of congenital origin, commencing early in foetal life, before the third month, when normally the septum closes. Hence patency of this septum furnishes in many cases a valuable means of determining the date of the primary defects with which it is found combined. This malformation, however, does very rarely stand as an isolated defect, and still more rarely it is believed to have been acquired through an ulcerative destruction (myocarditis) of a portion of the septum, either during foetal or extra-uterine life; wasting or atrophy of the membranous part is sometimes thought to have occurred. In these latter cases a misdirection of the blood-current of a marked sort rarely occurs unless the inflammatory or other changes affect the main arterial orifices.

5. The aortic and mitral orifices are very much less frequently found narrowed or obstructed as the result of congenital primary defects than the orifices and their valvular apparatus of the right heart; and, also following the rule which obtains on the right side, the mitral is less frequently affected than the aortic orifice.

6. Of the Malformations of the Great Vessels.--Such changes may come alone, though usually they are combined with simultaneous or consecutive defects in other parts of the central circulatory apparatus. Of the sorts of {691} defects or malformation which these two vessels suffer, there are two chief forms to be described: 1, such as result from an unequal division of the vessels in their formation from the truncus communis; 2, those which result in more or less complete transposition of their origins. Of the transpositions we may find two sorts: in one the vessels maintain nearly their normal relative positions to each other, but each communicates with the improper ventricle; in the other they are transposed relatively to each other and also to the respective ventricles. In the first of these classes, unequal division, one variety may be ascribed to a defective or irregular development of the septum by which the vessels are formed of unequal sizes; the other, originating later in foetal life--i.e. after the third month (for the septum between the vessels is completed nearly simultaneously to the ventricular septum)--results from inflammatory or other morbid change in or about the orifice and trunk of one or other vessel, causing a narrowing or closure, the other vessel showing compensatory enlargement. This form is not a true unequal division of vessels. The apparent origin of one or both vessels from the same ventricle in these cases is not a true example of transposition of the vessel, but is due to a deviation of the septum ventriculorum toward one side or the other from increased blood in the ventricle from which the outflow is more or less completely obstructed. True transpositions of the vessels, both relatively to each other as well as to the ventricles, originate very early in foetal life, and these as well as the unequally-divided vessels are primary defects, and are usually accompanied by many secondary changes. Another malformation occasionally found, involving the beginning portion of the great vessels, is a failure of complete division: the septum truncus communis remains rudimentary, and the blood of the aorta is free to mingle with that in the pulmonary artery. This defect may be accompanied with a rudimentary septum ventriculorum.

7. Ductus Arteriosus Botalli.--This foetal orifice varies greatly in the conditions which are presented; sometimes it is entirely wanting, in others patulous and even in a state of dilatation; in others, again, a short portion is patent (this state is probably comparable to failure to adhere seen in the valve of the foramen ovale or the sieve-like opening in the fossa ovalis; unlike the valve of the foramen, the ductus probably never reopens), or in yet others the ductus is closed in some cases of malformation, and in others of very similar character it remains open. It becomes difficult to explain the varied states of the duct, so dissimilar are they to other defects of development present. In none of the conditions which are presented can the malformations be regarded as of a primary character. Our surprise at certain of its conditions probably must depend on a failure to justly appreciate the primary malformation present, or else on changes in the heart and the circulation coming at a period subsequent to the date of origin of the malformation of the duct itself. When the duct is open at one end and closed at the other, the open part communicates usually with the pulmonary artery, since the closing process commences normally at the aortic extremity: the closure beginning at the pulmonic extremity is occasionally seen in malformations of heart where the blood-current has had a reverse direction through the duct.

The premature closure of the ductus arteriosus Botalli, which is spoken of by some authors, seems to be a rather unfair designation to apply to the condition. In most cases it is in reality an absence of the duct dependent on the defective development of certain of the branchial arches. In other cases the apparent premature closure is due to general uniform narrowing, almost closure, of the pulmonary orifice and vessels; in such cases the lungs are supplied by the enlarged bronchial arteries or other collateral branches. The ductus arteriosus Botalli remains patulous when the pulmonary artery is {692} narrowed or closed; in these cases the blood from the right side of the heart to reach the lungs must pass either through an opening in the septum ventriculorum or through the patent foramen ovale. The duct is generally open in cases of transposition of the main arteries, or even in cases of obstruction of the aortic orifice, or of uniform narrowing of the descending aorta or its main branches. Its usual length and its point of origin from the pulmonary artery or its branches, as well as its junction with the aorta, may vary. Two ducts have been found--one from each pulmonary branch, one of them joining the aorta as usual, the other seeking one of its branches. A distinct duct has been found arising directly from the right ventricle. None of these defects are to be considered as primary malformation, but as the secondary results from alterations of the circulation occasioned by other malformations of the heart or of its great vascular trunks.

* * * * *

Fully bearing in mind the distinction which must be made between primary and secondary defects, and the fact that congenital lesions of the orifices and valves are mostly located on the right side of the heart, let us look at various causes which are capable of producing malformations. In many cases, from the condition of the parts, it is possible to say positively that the alterations are dependent on an inflammatory process commencing in the endo-myocardium at an early period of foetal existence; this is true even after excluding cases in which the inflammatory products present may fairly be considered to be the result of defective development and not its cause. Inflammation was, as has already been shown, long ago pointed out as the cause of these obstructive malformations. Rokitansky (1844) was followed in his views by many, who asserted, probably wrongly, that this condition was the sole cause of the misdirection of development. It was considered that while in very many cases the evidences of the inflammation remained indubitable, in others, through a greater lapse of time, the inflammatory products became less distinct or were wholly removed. Thus, all defects of development may be traced as the results of some obstruction of the pathways of the foetal blood, which, on the one hand, effects the closure of certain vessels or orifices, or on the other hand maintains patent others which normally should be obliterated. It is much easier to trace these causes when they operate during the later periods of development, after the heart and great vessels have assumed the general shape they maintain, than those which operate at the earlier periods of transition. It is plain to us that an obstruction of the pulmonary artery or its branches coming before the end of the third foetal month must, by preventing the flow of blood through it from the right ventricle, maintain an opening of greater or less size in the incomplete septum ventriculorum. It is much less easy--or, in fact, impossible--to be positive about an obstruction or other change which causes the transposition or an unequal division of the great vessels, or which prevents entirely the development of either septum. Nevertheless, we can believe that some obstruction of the foetal circulation causes the former defect as well as the latter, if we may judge of so dark a question by the analogies. In fact, what would present itself as a trifling obstacle in the third or fourth month of foetal life would in the sixth week be an impassable obstruction.

It has been urged against the view that some inflammatory process is the invariable cause of the obstacle, by those who support the development theory, that, as the heart remains in a rudimentary condition, the defects result from a want of formative or plastive activity of the parts. It seems, however, as difficult to account for the want of formative activity which prevents the development of the septum or causes an unequal division of great arterial trunks as to find the traces of an obstruction. Maternal impressions or shocks have doubtless caused many headless {693} foetuses or otherwise misshapen the product of conception during the early months of development. The effect on the foetus from such shocks cannot of course be a direct nervous impression, such as those seen producing local disturbances of nutrition or of formative activity in the adult's own organism, but it is due to disturbances of the placental circulation, by which the blood-current is delayed in the foetal circuit. Such delay may result in a temporary obstruction of the blood in certain foetal vessels. A delay of the blood-current during a few hours in the early period of development of the foetus, when formation is excessively rapid, may result in changes which become permanent. The evidences of such obstruction may fade completely. Osler[7] has recently urged that it is difficult to suppose an endocarditis limited to the pulmonary valves in an embryo not more than an inch in length, and whose heart could not be above a few millimeters in size. But is it not possible to suppose an endocardial inflammation which affects at the same time, for example, the vascular orifices and the line of the rudimentary septum? The septum may thus be prevented from further development, and the orifice suffer malformation by subsequent contraction. The evidences of the inflammation would greatly lessen as the size of the heart expanded. Cannot inflammation, syphilis, or other communicable disease, from which we know the foetus suffers, be substituted for the unknown "want of formative activity"? In respect to the extent of surface involved in the foetal heart in inflammatory or other morbid processes, can we not suppose that the area exhibiting evidences of disease in the minute heart would be as restricted as in the adult heart? In rheumatic endocarditis of the adult the cause which leads to the inflammation is a general one; the evidences we find of the morbid process, however, are confined to very narrow limits. The reasons for this restriction may be the same.

[Footnote 7: Montreal Gen. Hos. Reps., vol. ii.]

The simple narrowing of a blood-track where direct evidence is wanting may be explained by the occurrence of a specific morbid process as satisfactorily as by an appeal to lack or excess of formative power. The real difficulty arises in the explanation of cases of transposition of the great vessels. The problem is in every way a most difficult one for solution under any supposition. If it were true that the formation of the pulmonary artery and the aorta was from the start by separate blood-channels, and these distinct vessels suffered a genuine transplantation and became attached to the wrong ventricle, the aorta to the right and the pulmonary to the left ventricle, then undoubtedly we should be compelled to accept the developmental theory as usually expressed. But it is not the case that these vessels are developed in distinct trunks: their development results from the division of a common trunk through an infolding of the walls or the gradual formation of a septum proceeding contemporaneously with the septum of the ventricles, the vessels at the same time making a half turn on their axis. A delay in the formation of either septum may result in the malapposition of the vessels to the ventricles. The septum which is probably delayed in formation is the vascular septum, since it is apparently the growth of this septum that applies the force which results in the axis rotation of the vessels. Are we again to explain the abortive formation of the vascular septum or any portion of the branchial arches by the unknown want of formative power? The want of formative power must have a cause; it does not come spontaneously. Are not inflammatory endarteritis and syphilitic lesions of the blood-channels probable causes of the contraction or obliteration of portions of the branchial arches?

Another question, dark and obscure, requires a short comment. It is commonly accepted, if an abnormal communication (speaking of small openings) exists between the two ventricles, that the septum has been prevented from {694} closing by the blood-current being diverted from its usual course through narrowing of an arterial ostium, and compelled to flow into one or the other ventricle. The patency or the closure of the ventricular septum is held as a criterion of the date of origin of the primary malformation. We know that certain ulcerations of the endo-myocardium may result in forming openings between the two ventricles, but is it not possible that a perforation may be made in the ventricular septum after it has closed by a lesion originating at an arterial ostium of the same character as one that prevented the septum from closing? The muscular tissue of the heart from the third to the sixth foetal month, and even later, is of very soft character. A rapidly-coming closure, or even temporary obstruction, of one or the other great arterial trunks would greatly increase the blood-pressure within the corresponding ventricular cavity. The ventricular septum would become stretched and thin, and might readily be perforated, so delicate is the muscular tissue.

If such a possibility is consummated, it must alter the value which has hitherto been placed on the opening in the ventricular septum as a criterion of the date of origin of the primary lesions of the great vessels which ordinarily are the cause of the patent condition of this partition.

* * * * *

It is to be seen from a review of the recorded cases of malformation of the heart that defects of the arterial outlet of the right ventricle are the primary cause of the largest number of cases. It is impossible to state the proportion of these to those at other orifices or the great vessels, so incomplete are the records and so unlike are the opinions of the reporters. It is but natural that this the more active ventricle of foetal life should exhibit more frequently defects of development, since the left ventricle in adults suffers more commonly in its valvular apparatus during its more active period.

The position at which the defects resulting in obstruction of the blood-current through the pulmonary artery may occur have been mentioned. The degree of the narrowing is of much importance--much more than the seat of the obstruction; but of still greater consequence is the date of origin of the defect of development, since on its occurrence early or late in foetal life depend the condition of the septum ventriculorum and the perfection of secondary compensatory alterations which render the heart capable or incapable of a prolonged extra-uterine life.

Narrowing or closure of the course of the blood passing through the pulmonary artery may be divided into two classes: 1, those cases in which the septum ventriculorum is imperfect to a greater or less degree; and 2, those in which it is fully formed, the separation between the ventricles being complete. The date of their origin corresponds to different periods of the development of the foetus. The earlier the obstruction comes in the normal outlet of the ventricle, the more rudimentary is the ventricular septum. The size of the opening of the septum depends on the degree of narrowing of the pulmonary outlet as well as on the date of origin of the obstruction. If the arteries are transposed in relation to the ventricles, and one of them becomes obstructed, the effect on the septum is the same, although the direction of the current through the opening is reversed. Kussmaul and others have pointed to certain exceptions which may lead to errors. In a congenital opening of the ventricular septum, isolated from other defects, an endocarditis involving the pulmonary orifice may occur subsequent to the time of the usual closure of the septum, or even after birth. It would be difficult to distinguish such a case from one of pulmonary narrowing occurring before the third foetal month. The character of the inflammatory changes and the size of the pulmonary artery beyond the point of narrowing would assist in marking the distinction. It must be remembered, however, that the pulmonary artery is {695} recorded as possessing a large size beyond the seat of narrowing in cases of undoubted congenital origin.

The alteration in the form and size of the right ventricle varies greatly according to the time at which the pulmonary obstruction originates. The ventricle seems to maintain its size, and even to become hypertrophied and dilated, when the pulmonary obstruction occurs before the closure of the septum: if the pulmonary artery is obliterated or exceedingly narrowed at a later period, the ventricle shrivels, because no blood is able to pass, and gradually more and more of the foetal current passes through the foramen ovale to the left side; if, however, the pulmonary defect is but slight, the right ventricle continues its function, becomes hypertrophied, and may dilate. In pulmonary obstruction the right ventricle changes its form somewhat in accordance with the seat of obstruction. Thus the primary obstruction may be in the pulmonary artery or its branches; or in other cases the malformation is found within the cavity of the right ventricle. The last group is spoken of as conus stenosis.

The malformations of the conus of the right ventricle may present themselves under three forms: they all act as constrictions, but alter the shape of the ventricle very variously; their effect on the circulation is practically the same, varying only with the closeness of the constriction. If an inflammatory process occur at the seat of the normal muscular constriction between the sinus and the conus, it may result in fibrous thickening and contraction; thus the normal division of the sinus from the conus becomes exaggerated and permanent. The narrowed portion may continue to exhibit evidences of endocarditis, or these may fade away, leaving a smooth surface. These narrowed parts seem to be especially liable to inflammation at a subsequent period as the bulk of the blood and the force of the circulation increase. Peacock describes a condition of narrowing due to muscular hypertrophy alone. It would seem in these cases that the hypertrophy was, in not a few of the instances, an acquired condition, and not congenital.

These cases present a heart having, as it were, a double or subdivided ventricle, comparable to that of the turtle. The condition has been described by some writers as a supernumerary ventricle. The form and size of the communication between the two portions of the ventricles vary very greatly: in some of the cases due to inflammation the passage merely admits of a large probe, and consists of a firm fibrous ring, or there may be two or more such openings. In constriction by muscular bands the opening is usually a large oval with smooth walls. In these cases the size and the condition of the walls of the so-called supernumerary ventricle present different appearances according to the degree of constriction and the size of the pulmonary opening; it is probable also that the condition of the ventricular septum influences the consecutive alteration in the parts. When the constriction is close and but little blood enters the conus, its walls are thin and flaccid, while in cases of less marked narrowing, provided the pulmonary artery remains nearly normal, the walls of the conus become hypertrophied, in conjunction with a similar development of the other parts of the right ventricle.

In other cases the entire conus may be uniformly narrowed: this change is due almost invariably to inflammatory lesions, and in many instances it is difficult to determine whether the condition is of foetal origin or whether it arose during the early months of extra-uterine life or even at a later period. Its occurrence in conjunction with other malformations would point to its origination during the developmental period. The conus may also present a constriction directly at or just beneath the valvular orifice of the pulmonary artery. This condition is almost invariably combined with some narrowing of the artery itself, and there is so constantly present evidence of inflammation of recent date that it is almost impossible to say whether the defect {696} is not due to a myocarditis originating after the developmental period. With this condition the entire conus usually presents more or less shrinkage or collapse, becoming greater as the constriction at the orifice is more marked. This collapse of the conus is to be looked upon as secondary to the primary defect at the orifice.

Closure or narrowing of the pulmonary artery trunk may be traced to many conditions acting at several different points of the course of the blood. Nearly all these conditions are caused by inflammatory lesions which result in contractions of the arterial walls. In fact, pulmonary artery defects not dependent on inflammatory changes are very obscure and difficult of explanation. In adult life we know of only two conditions which lead to obliterations of vessels; first, inflammation of the lining membrane (endarteritis); and second, stoppage of the blood-current, usually through pressure directly applied to the vascular trunk. The clots of blood which occupy the vessels form both in advance and beyond the point of pressure; hence we can look for obstruction, causing closure of the pulmonary artery, at either extremity of the blood-course. Thus, we may think of a primary conus obstruction which may secondarily have the effect of reducing the size of the pulmonary artery, but it is never obliterated through this means; nearly always some blood passes in this direction, and blood also enters the pulmonary artery from the ductus arteriosus Botalli: both conditions necessarily tend to keep the artery from complete collapse; moreover, the artery, even in cases of very narrow conus, may remain of its usual size. The same effect may be produced by narrowing of the tricuspid orifice. This condition is a very rare one, and never could lead to complete closure of the pulmonary artery unless this orifice were entirely obliterated and the septum of the ventricles remained closed. Peacock speaks of premature occlusion of the ductus arteriosus Botalli as one of the causes of narrowing of the pulmonary artery. The obliteration of this portion of the branchial arches, by preventing the blood flowing in its usual course to the descending aorta, he thinks results in narrowing the calibre of the pulmonary artery. May not the condition be equally well interpreted in a different manner? May not it be that the obstruction of the artery was the cause of collapse of the ductus? One would think it possible, if an obstruction arose in the ductus arteriosus Botalli, for the blood-current in the pulmonary artery to maintain another branchial arch patulous for its accommodation, or, failing this, to dilate the pulmonary branches and thence return to the left side of the heart. In rare cases the pulmonary artery has been found deficient in size when the lungs are malformed, either by reduction in their size as a whole or by the absence of one or more lobes. Such a cause has very little opportunity of acting with much force on the pulmonary artery during foetal life. This cause and all the others in this group are to be looked upon as secondary in their effects.

In primary defects of the pulmonary artery trunk the vast majority afford indubitable evidences of an original inflammatory causation; others are due just as positively to a defective evolution of this vessel from the common arterial trunk. Instances are on record of the complete closure of the pulmonary artery and its conversion into a ligamentous cord: these cases are very rare. In a somewhat larger number a pretty uniform narrowing, sometimes to an extreme degree, and often exhibiting thickened walls, is found. It is much more frequent to see the obstruction of the artery, due to inflammatory changes, at its valvular orifice.

Peacock describes the narrowing at the pulmonary orifice in many cases to be due to disease of the pulmonary valves, whereby the number of cusps are reduced in number, or to a membrane stretched across with small openings in its central portion; or the obstruction may consist of a duplicature of the lining of the vessels, or even to bands of muscular fibres surrounding the {697} orifice. Two valves of unequal size may be found at the orifice, giving evidence that the larger one has been formed by the adhesion of two of the normal cusps; the membranous obstruction is probably due to the union more or less complete of the three cusps. The curtains thus formed protrude into the course of the artery and form a deep circular sinus between the valves and the walls of the vessel. The opening between these adherent valves varies from a transverse slit to a tubular or barrel-shaped orifice--a tube within a tube. These diseased valves are thickened, very firm, fibrous, or even calcified. In other cases the obstruction consists of abundant warty elevations, so numerous that they are equally effective in preventing the passage of blood as the united valves. The size of the opening is sometimes extremely reduced, measuring only five millimeters in diameter. The pulmonary artery is most generally less in size than normal, but never becomes reduced to the same extent as its orifice, unless it has likewise suffered from inflammatory disease; otherwise its walls remain thin, resembling the venæ cavæ.

In addition to disease within the calibre of the vessel, Meyer, who strongly advocated the inflammatory cause for all these defects, pointed to pericarditis, occurring at the origin of the pulmonary artery and compressing the vessel, as a rare method of causation.

In a very large majority of the cases of pulmonary narrowing on record the septum ventriculorum is found to be more or less defective. In accordance with the usual principles, this defect of the septum, in conjunction with narrowing of the pulmonary artery, is held to indicate that the obstruction of the artery dates from a period of development anterior to the closure of the septum. This view was advanced by Hunter in 1783. But Peacock gives an account of many cases of pulmonary narrowing, combined with open septum ventriculorum, in which the obstruction was caused by adhesion of the pulmonary valves. It is, however, a fact that the development of the valvular apparatus is not effected until after the septum of the ventricles is completed. How, then, can we suppose valves to adhere so as to obstruct the pulmonary artery and prevent the closure of the septum when in reality the valves themselves have not developed? Does it not seem possible that in some rare cases the opening found in the septum ventriculorum is in reality a reopening? Another case is on record of open septum ventriculorum and narrowing of the pulmonary orifice in a child born of a mother who suffered a prolonged fright during the fifth month of utero-gestation. Strong mental impressions are accounted causes of malformation of the foetus, and in this case the fright, if it was the origin of the defective development of the septum, came more than two months too late.

In cases of pulmonary narrowing with open septum the aorta communicates freely with the right ventricle, or appears to arise from both ventricles, or more rarely from the right cavity alone (the deficient pulmonary artery remaining in its usual position). Many opinions have been held as to which one of the three defects is primary. Hunter's conclusion has most generally prevailed. The obstruction of the course of the pulmonary artery is looked upon as the primary defect. From the obstruction the right ventricle becomes distended, and the opening of the septum is due to the blood-pressure, which prevents the final closure. The blood-pressure also alters the direction of the septum and pushes it farther to the right. Thus the septum comes to stand directly under the aortic orifice, or by a further deviation to the left side brings that orifice wholly within the right cavity. In these simple cases the origin of the aorta from the right ventricle is not a real but merely an apparent transposition or transplantation of this vessel; the aorta has not been moved, but only the septum has been moved under its orifice, and the right ventricle has consequently become more extensive. In other cases the aorta {698} seems to move more toward the right side, usually coming also more to the front, and in other cases there is an actual transposition of these vessels. The method of this transposition will be further described.

Meckel's original theory for open septum and narrowed pulmonary artery was that the defect was primarily in the septum of the ventricles, due to a want of formative energy, and the pulmonary artery closed itself, as do other arteries, from want of use. Meyer showed that a defect of the septum was incapable of causing narrowing of the pulmonary artery, since the exit of blood is easier through the artery, from the form of the right ventricle, than through the open septum; the passage of the blood from right to left is opposed by the blood-mass in the left cavity. Heine also thought the pulmonary-artery narrowing was a secondary defect, but did not think the opening of the septum caused the narrowing. He considered the primary malformation to be a deviation of the septum to the left. The deviation of the partition before its closure brought the aorta within the left cavity, and furnished a free exit for the blood from this chamber shorter and more convenient than through the pulmonary and the ductus arteriosus Botalli to the descending aorta; the pulmonary artery collapsed for want of use, similarly to other foetal blood-courses. Hence, Heine considered that in all cases of open septum and apparent transposition of the aorta which exhibited no evidences of inflammation as a conjectural cause of narrowing or closure of the pulmonary artery the explanation was to be found in a primary deviation of the septum ventriculorum.

The difficulty in Heine's theory lies in showing the mechanism of a deviation of the septum without a primary obstruction of the flow of blood through the pulmonary artery. The hypertrophy of the right ventricle which Heine proposed as an explanation is almost certainly a secondary effect of the obstruction, and therefore cannot be supposed to originate a deviation of the septum; it is doubtful if hypertrophy can be considered as a cause of increased blood-pressure within the cavity of a ventricle under any circumstances, and certainly not as exercising pressure in a direction to cause the supposed deviation of the septum. An open septum without obstruction of the pulmonary orifice, which rarely occurs, does not produce hypertrophy of the right chamber.

The explanation of cases of open septum with obstruction of the pulmonary artery seems entirely satisfactory by Hunter's theory, or by what Kussmaul has named the engorgement theory. But when there is a real transposition of the arteries, the pulmonary placed farther to the left and behind and coming from the left cavity, the aorta in front and to the right and arising from the right or pulmonary chamber, thus changing their relative positions and their orifices exchanging ventricles, the difficulty of explanation becomes great, and the cause of the abnormal relations of the vessels cannot be traced to a simple deviation of the septum ventriculorum.

For the explanation of these cases of complete transposition of the vessels, as well as their transplantation relatively to the ventricles, Rokitansky has traced respectively the development of the two arterial trunks from the common trunk and of the septum ventriculorum. He considers that the partitioning of the arterial trunks is the governing factor in their formation, and that the ventricular septum is arranged in conformity with the septum of arterial trunks. In tracing the development of the circulatory apparatus in man there seems to be no doubt that the heart develops exactly like that of other vertebrates.

The very first rudiment of the heart is a spindle-shaped thickening of the intestinal fibrous layer of the fore part of the alimentary canal. This spindle-shaped formation then becomes a hollow pouch, and separates from the intestinal layer and lies free in the cardiac cavity. The earliest condition {699} yet seen in the human being is that from an embryo of about two weeks (Coste), in which the viscus appeared as a simple tube in the shape of a letter S--the hollow rounded pouch having slightly elongated and bent to this form, and simultaneously turned spirally on an imaginary axis, so that the posterior part of the tube rested on the dorsal surface of the anterior part. The yelk-veins connect at its posterior part, while the arteries form a continuation of its anterior extremity. The spiral turning and curving increase, and simultaneously two shallow indentations appear in the twisted pouch, transversely to its long axis, looking like kinks in a flexible tube. These indentations mark the outline of the three primitive portions of the central organ--viz. the first, with which the veins communicate, represents the future auricles; the next, the ventricles; the third portion, the common arterial trunk (aortic bulb or truncus arteriosus communis). Early in development the first section is the largest, but by the time the S is formed the middle or ventricular portion exceeds in size the auricles and their appendages. So far, the central organ remains a continuous tube, indented transversely in its course at the points which mark its future division; the blood moves through it as through a coiled tube, entering by the veins and passing out by the aortic bulb to the vascular or branchial arches; the venous entrance is posterior, the arterial exit is anterior and is directed toward the future aortic arch. This is the condition at the end of the second week. The future auricles and ventricles now form a common cavity; the indentation between them, called the auricular canal, represents the future auriculo-ventricular orifice. The future fibrous ring forming this orifice is the first to be developed of all the permanent structures of the heart; its infolding to form the two auriculo-ventricular orifices comes early, but at a later date than here spoken of. Its exact method of development is not clearly described.

Between the second and fourth weeks is exhibited an indication of the future most important step in development; this process does not really step forth until the fourth week, although superficial traces of a furrow antedate this time. This step is the division of three sections of the tube into opposite halves, a right or venous, a left or arterial half. This division results in the formation of the future septa between the auricles and between the ventricles, and separates the common arterial trunk (aortic bulb) into the future aorta and pulmonary artery. This partition is spoken of as longitudinal; but it will be seen, if the real lines of growth of the future auricular and ventricular septa are carefully regarded, that the indentations which mark their site are also transverse, as were the primitive ones for division of the auricles from the ventricles. The proximal end of the tube comes in contact with the distal portion by a further bending movement, so that these two ends go to make the left half of the heart; and the middle portion of tube, composed partly of auricle and partly of ventricle, forms the right half of the heart. This secondary indentation, commonly spoken of as longitudinal, is in reality transverse, although, from the more markedly bent condition of the tube which has come about, it does not advance in the same plane as the primitive indentation of the tube. The mechanism of the division of the aortic bulb will be described later.

This secondary indentation, which finally results in the formation of the auricular and ventricular septa, appears earlier in the ventricular cavity, about the fourth week, and later in the auricles, about the eighth week. By about the twelfth week the process of formation for the muscular partitions is completed; the septum ventriculorum normally is gradually built up, and by this time has joined itself, at the base of the heart, to the septum forming itself in the arterial bulb; thus the right and left ventricles are finally separated. The septum in the auricles is also finished in its muscular part, mostly built up from the base and posteriorly toward the roof of the cavity, {700} leaving, however, the foramen to be closed by the membrane some days after birth.

The foetal heart from the fourth week onward becomes more and more rounded in outline, and finally more or less rectangular. The auricular appendages become conspicuous and overhang the ventricles. The future left ventricle appears larger than the right, and the former projects notably leftward and downward. The aortic bulb or common trunk appears to arise wholly from the right ventricle, although the vessel communicates with both cavities, since at this period the cavities are undivided. The furrow which marks the line of the future septum ventriculorum runs to the left of the root of the common trunk; and until at least as late as the sixth week this trunk appears from the exterior to be in connection only with the future right ventricle.

As early as the sixth week, possibly earlier, a distinct furrow is seen on both sides of the common trunk running longitudinally from its root at the ventricle to its first branch (branchial arch). This indentation does not traverse directly to the ventricular furrow; in fact, at this period the ventricular furrow is not conspicuous at the origin of the trunk toward the base of the heart, the septum within not having risen as yet to the base of the ventricles. During the formation of this furrow the common trunk continues its slow partial rotation on its axis; the rotation of the other parts of the cardiac tube has ceased; the segments of the tube have come to a standstill--become, as it were, fixed and adherent to each other, the proximal to the distal end, the anterior surface to the posterior, through the previous bending of the tube on itself.

Within the common trunk Rokitansky has described the changes, as seen in cross-sections, which result in its division into a permanent aorta and pulmonary artery, and also the adaptation of the septum arteriosus trunci to the septum ventriculorum. He says that at an earlier period than here described for the external furrow appearing, on the inner surface of the truncus arteriosus communis (aortic bulb), to its left side and somewhat posteriorly, above the starting-point of the anterior limb of the septum ventriculorum, a little swelling appears, which grows toward the right and slightly forward, so that the common trunk is divided into an anterior rather left-hand, and a posterior right portion, respectively the pulmonary artery and aorta. The growth does not pass in a straight line through the lumen of the common trunk, but so that the forming septum makes a concavity posteriorly toward the aorta, and a convexity anteriorly toward the pulmonary; thus, on cross-section the aorta has the outline of the gibbous moon--the pulmonary, fitting into it, separated by the septum, of a new moon. The septum ventriculorum, as seen starting at the base of the ventricles from the fibrous ring of the auriculo-ventricular orifice (having already been built upward from the future apex of the heart), originates at a point on the posterior wall of the common ventricular cavity in exact correspondence with the starting-point of the little swelling on the inner surface of the common arterial trunk. The two septa are thus formed in apposition. The septum ventriculorum, in advancing forward to meet the other limb of the septum forming on the opposite wall of the ventricular cavity, follows the septum trunci arteriosus communis, surrounds the posterior vessel (the aorta) to its front, then passes around it to its right; the pulmonary is on the other side of the septum; the portion of the septum ventriculorum between the orifices of the vessels is the pars membranacea of the septum. The anterior portion of the septum ventriculorum forms one wall of the arterial conus of the right ventricle. Thus it happens that by the eighth week the common trunk is divided into aorta and pulmonary artery; the structure of the septum ventriculorum is so far advanced that these vascular trunks are connected with the proper ventricles, but the {701} septum ventriculorum does not close completely until about the twelfth week.

In explaining the occurrence of a transposition of the arterial trunks in accordance with the facts of their normal development, Rokitansky says, if the septum trunci, starting from the usual point of the little swelling on the inner surface of the common trunk, turns abnormally with its concavity forward (instead of backward as normally), and thus passes through the trunk, there will be established an anterior left aorta and a posterior right pulmonary, because the septum ventriculorum in its growth conforms to the direction of the septum trunci. Thus, another than the usual portion of the common trunk is partitioned off and placed in communication with the respective ventricles. This furnishes us with examples of transposition of the arterial trunks relatively to each other, but not transposed in relation to the ventricle into which they are implanted. The great majority of specimens of this sort with which we are acquainted--and Rokitansky knew no others--show an open septum. They are usually spoken of, therefore, as instances of "both vessels arising from the same ventricle (the right usually)," or of "aorta communicating with both ventricles, the pulmonary artery normally placed." Rokitansky assigns no reason for this deviation in the line of growth of the septum trunci across the lumen of the common trunk; in fact, he never examined a malformed heart during this stage of development. The deviation of the septum trunci, the primitive factor in this malformation--since to it the septum ventriculorum conforms its development--he accounts for by chance (deviation of formative energy). It seems much more probable, as it is always the pulmonary artery which must be reduced in size when the concavity of the septum trunci presents anteriorly (the aorta occupies the smaller area when the concavity of the septum is posterior), that the deviation of the septum trunci is due to some one of the many conditions (endo-myocarditis) which have already been pointed out as the cause of pulmonary-artery narrowing or closure; hence, another malformation of the heart can be thus traced to pulmonary obstruction, the evident cause of so many other defects.

For examples of transposition of the vessels, both relatively to each other and to the ventricles, with complete closure of the septum ventriculorum, Rokitansky also gives a satisfactory explanation. It is important to note the distinction between cases of closed and open septum. Transposition of the vessels with open septum are, as already shown, doubtful instances of transposition from one ventricle to the other, although the vessels may be transposed in relation to each other; furthermore, the mechanism which explains relative transposition of the vessels does not explain the implantation of the vessels into the improper ventricle. His explanation is that the starting-point of the little swelling from which the septum trunci forms is shifted to a point farther forward on the inner circumference of the common trunk, and at the same time has its concavity anteriorly, and as in the previous case decreasing also the area of the pulmonary artery; and thus the aorta comes more forward and to the right, and the pulmonary artery passes more to the left and backward. The septum ventriculorum, in conforming itself to the abnormal starting-point and direction of the septum trunci, must consequently pass across the common ventricular cavity in such direction that the aorta comes in connection with the pulmonary side of the heart, and the pulmonary artery with the systemic heart. Consequently, Rokitansky traces both the relative and the actual transposition of the arterial trunks to the deviation either of the direction or of the starting-point of the septum trunci. The deviation of the position of the little swelling on the inner surface of the common trunk, which Rokitansky supposes, is probably not an actual transference or misplacement of this point of formative energy, but in reality a failure of the common trunk (aortic bulb) to continue its axis-rotation, as it {702} normally does, after the other portions have become fixed. This premature cessation of the rotation of the common trunk would leave the starting-point of the septum trunci in a more anterior position than normal, since the trunk rotates normally in a direction to bring its left side, on which the starting-point of the septum trunci is situated, more posteriorly. A pericardial inflammatory adhesion, such as Meyer pointed out for certain cases of pulmonary artery obstruction, would fix the common trunk, prevent its proper rotation, and at the same time narrow the pulmonary orifice in certain instances. In other cases, in which the pulmonary artery is found of normal size, the septum trunci may be supposed to divide the vessel in the usual direction (concavity posteriorly as normal), whilst the septum trunci commenced to grow from an abnormal position, more anteriorly and to the left than normal (through failure of rotation); hence, as the septum ventriculorum conforms to its growth, the vessels become connected with the improper ventricle; the pulmonary, however, is not found permanently narrowed, and the septum ventriculorum is completely closed. Here the cause is a failure of the common trunk to rotate on its axis, probably from an external adhesion of its periphery.

Malformations affecting primarily the Right Side of the Heart.

In classifying defects in the course of the pulmonary artery we come to--

1. Closure or narrowing of the artery, with perfect ventricular septum.

Congenital obstruction of the pulmonary artery, with closed septum, although more rare than with open septum, is nevertheless a frequent defect. Unfortunately, it is very often impossible to distinguish with certainty whether the stenosis is essentially congenital or is acquired after birth. Complete closure is the least difficult to distinguish, because this defect very soon causes death; the prognosis in a merely narrowed orifice is much more favorable. The duration of life in complete closure never extends beyond a full year, while in undoubted congenital narrowing the age of sixty-five years has been attained.

From this atresia the most striking consequence is a reduction of size of the right ventricle, increasing almost to closure. This result is so common that Peacock thought it was the law that in atresia the right ventricle reduced itself to closure, while in stenosis it dilated and became hypertrophied. This is not the law, but only a rule of very common occurrence. Instances of eccentric and concentric hypertrophy are found among the records of these cases. Great reduction of the right ventricle results probably only when the obstruction comes very soon after the completion of the septum ventriculorum--thus at a time when the ventricle is yet very small. The wasting of the right ventricle can reach a very high degree, and when it becomes very great the tricuspid orifice is also defective. The foramen ovale and the ductus arteriosus Botalli are, in complete closure, usually found open. The obstruction may come in the conus or at the valvular orifice, or the artery is found converted into a cord.

In seven cases the duration of life varied from four days to nine months.

When the stenosis does not reach a high grade, positive clinical signs are often wanting for the determination of its existence, and the difficulty becomes greater as the age of the person advances.

Clinically, we find congenital blueness with palpitation, dyspnoea, together with the physical signs of pulmonary stenosis; these symptoms, however, may make their first appearance only on the advent of some acute disease. Sometimes they come in the first month or the first year of life, or even much later. {703} If abundant congenital compensatory changes are present, the symptoms may be postponed until further compensatory alterations become impossible; or at the narrowed orifice the development of a fresh endocarditis may determine the occurrence of symptoms. The mere increase of the body and of the mass of the blood may alter the relations of the circulation, and this disproportion may show itself with suddenness. Febrile conditions may also suddenly disorder the circulation.

The compensatory alterations which commonly are held to indicate a congenital origin of stenosis of the pulmonary artery are patulousness of the auricular septum and of the ductus arteriosus Botalli. When both of these remain open there cannot be much doubt that the date of origin is from the foetal state or in the first weeks of life. If only one of the foetal passages remains open, the ductus gives a greater surety of a foetal date than the foramen ovale. The closure of both foetal passages does not exclude a congenital origin if the obstruction of the pulmonary orifice is moderate. The patulousness of both foetal passages indicates that the defect arose at least shortly after birth, because these openings close within four or five weeks of this event. The foramen ovale alone open indicates very little with certainty, as it is so often found with one or more small openings without any probable cause. Bézot found it partially unprotected in 44 cases out of 155; Klob, 224 in 500; Wallmann, 130 in 300. Rokitansky has indicated that a strong blood-pressure not unfrequently may press the fibrous valve of the foramen strongly toward one auricle or the other, and thus lead to its atrophy in part, forming larger or smaller openings of communication between the two cavities. In doubtful cases of stenosis of the pulmonary artery such small openings are not signs of much value in determining the congenital origin of the stenosis. Unless there is a marked defect in the septum atriorum, the congenital origin of the pulmonary atresia or stenosis cannot be predicated on this ground.

Patency of the ductus arteriosus Botalli has been very rarely observed as a primary malformation. A coincidence of this as primary defect with post-natal stenosis of the pulmonary artery must necessarily be extremely rare. In persistence of the ductus art. Botalli the current passes from the aorta toward the pulmonary artery; the obstruction of the pulmonary artery conditions a dilatation with hypertrophy of the right ventricle. Reopening of the closed ductus is impossible.

The condition of the pulmonary valves as well as the diameter of the pulmonary artery itself and its branches often afford valuable points for the determination of the congenital origin of stenosis of this orifice. The greater the narrowing, or the more extreme the thinning of the wall, the earlier the coming of the obstruction.

Morgagni reported the first case of stenosis of the pulmonary orifice--in fact, the first case of congenital malformation of the heart--in a girl aged sixteen. He recognized the relationship of the open foramen ovale and the dilatation of the right ventricle as mechanical effects of the pulmonary stenosis.

2. Obstruction of the conus arteriosus dexter, with open ventricular septum.

A. The separation of the conus in the form of a so-called supernumerary third ventricle has been reported by Peacock in ten cases, and ten others have been added by Kussmaul from various sources. The degree of separation varies very greatly in individual cases: in some it is so slight that the designation becomes doubtful, while in others it is so great that the word stricture might with propriety be employed. In two cases a goosequill-sized opening existed between the sinus of the right ventricle and the conus; in others the communication between the two was multiple. The size of the supernumerary ventricle varies greatly; in one case of a girl of twelve years it would only contain a hazelnut.

{704} In most of the cases the partitioning probably commences by hypertrophy of the muscular bands which are more or less marked in normal hearts in this situation: to this, as the result of endo-myocarditis, is added cicatricial contraction of the inflammatory products, whereby the original partitioning becomes greatly increased. The preponderating frequency of the seat of the stenosis directly at the transition of the conus to the sinus increases the likelihood of this explanation of its causation.

Alteration of the valves of the pulmonary artery, probably of foetal inflammatory origin, is of very frequent occurrence with supernumerary ventricle and conus stenosis; sometimes only two cusps are found, though four cusps have been noted; they may be absent or be replaced by a ring mass formed from their union. Normal valves have been observed unaltered by inflammatory changes. The conus appears always to suffer diminution, and the pulmonary artery is found more or less narrowed according to the degree of obstruction. The sinus of the ventricle is dilated and its walls hypertrophied. The aorta, mostly widened, springs in all cases from both the ventricles, unless wholly from the right one. The foramen ovale is generally more or less widely open, although it has been found closed; the ductus art. Botalli is mostly closed. The duration of life may be long; Kussmaul reports the oldest case at thirty-eight years.

B. Of primary uniform narrowing or shrinkage of the right conus art. dext. Kussmaul reports eight cases from various sources. The conus was shortened, and formed a ring-shaped fissure, gradually reduced in size toward the orifice of the pulmonary artery. The pulmonary valves were variously changed, mostly by union of one or more of the cusps, though sometimes remaining normal in shape, though very delicate. The pulmonary artery was generally narrowed to about the width of the calibre of the conus itself, unless further change came to it from its special involvement by endarteritis.

In all cases the aorta arose from both ventricles. The right side of heart was dilated, and the right ventricular wall hypertrophied. The foramen ovale remained open. The ductus arteriosus Botalli has been found absent or closed, and the collateral circulation effected by anomalous communications, oftentimes duplicate or manifold; in most cases the ductus remains open. The oldest patient in which this form of heart has been found was twenty-five years.

C. Ring-formed narrowing of the conus, due to a muscular band. Peacock reports this defect from a girl æt. nineteen, cyanotic from birth; the constriction was situated at the bases of the valves, and was formed by a muscular band covered by fibrous tissue, and the edges of the opening were studded with warty vegetations. The pulmonary valves were two in number, probably resulting from fusion of two of the cusps; were thickened and opaque, but smooth. The index finger could be passed between the valves. The artery was of small size, but much wider than the constriction. The aorta was dilated, and arose from both ventricles through a perforation of the septum ventriculorum. The foramen ovale was closed. The ductus art. Botalli gave free passage to a crowquill. The right side of heart showed dilatation and hypertrophy of both its cavities, and the tricuspid valve was thickened and had vegetations on its auricular aspect.

3. Simple stenosis and atresia of the pulmonary artery, with open septum ventriculorum.

This class includes malformations, with stenosis or atresia of the pulmonary artery, in which the defects occur before the completion of the ventricular septum, as the result of engorgement already described, but in which no other primary congenital defect exists; thus the separation of the pulmonary artery from the truncus arteriosus communis is completed; the auricles and ventricles are marked out by their septa, though not completely divided; {705} the position of the aorta in relation to the pulmonary artery is either normal or more to its right; and there are no primary defects of any consequence in the other orifices of the heart. The simple stenosis or atresia of the pulmonary artery as thus defined is by far the most frequent malformation of the heart. Kussmaul has found about 90 described; among these are 26 cases of atresia.

As a rule, in partial obstruction the entire length of the artery, as far as the bifurcation, shows narrowing, but the greatest narrowing exists at the orifice of the pulmonary artery; only rarely are the orifice and the tube equally narrowed. Exceptionally, the tube has been found narrower just without the orifice, and later resumed its normal circumference. The walls are very often thin, like those of veins, and at times the vessel is shrunken. The valves are variously altered, often to a greater or less degree united, thickened, and opaque.

In complete closure two different conditions are seen; in some the artery itself to its bifurcation changes to a firm cord or thread; in others the tube is more or less narrowed and the orifice alone is closed.

As a rule, in stenosis and atresia of the pulmonary artery the conus is only moderately narrowed and its walls hypertrophied, while the sinus of the right ventricle is dilated and hypertrophied. The right auricle is dilated and hypertrophied. The tricuspid leaflets are clouded and thickened. The left ventricle is commonly small, and the wall not thicker than the dilated right ventricle. Sometimes the aortic and mitral valves suffer alterations of an inflammatory sort, probably of foetal origin. The shape and position of the heart are changed, but the size, as a whole, may not be much altered. The aorta may be widened, often to double the normal size. As to the origin of the aorta, it is often difficult to speak with certainty; its relative position to the pulmonary artery and to the body and axis of the heart is, as a rule, unchanged. Whether it is to be described as arising from one or both ventricles, or from the right one alone, depends on the posture which the septum ventriculorum assumes beneath its orifice. As a matter of fact, this relationship makes little difference to the flow of blood from the right ventricle, whose normal orifice is obstructed; provided the septal opening is sufficient, the flow of blood is secured and the hindrance to the circulation precluded. The opening in the septum ventriculorum may be only at the membranous portion, or it may also involve the adjacent muscular septum; the defect may be round or triangular, with its apex above and with smooth margins.

The foramen ovale has been found open in 39 cases out of 53. Its condition in this respect shows very great proportional variation in the different collections of cases. The open or closed condition of the foramen does not seem to depend on the degree of stenosis of the pulmonary artery itself. It depends, probably, more on the freedom of escape for the blood from both the ventricles through the aorta--probably also on the condition of the ductus arteriosus Botalli. The foramen ovale and ductus art. Bot. have been found closed much more frequently in stenosis than in atresia of the pulmonary artery, and the ductus is deficient or absent oftener in stenosis than in atresia. This absence of the ductus occurs in 13 per cent. of the cases, and tends to support Peacock's theory that narrowing of the pulmonary artery is the consequence of the defective development of that branchial arch out of which the ductus art. Botalli is formed.

It is of great interest to note the collateral circulation by which blood reaches the lungs when the pulmonary artery is closed. When the ductus arteriosus is open, the blood passes from the aorta into the ductus and the branches of the pulmonary artery become branches from it. When the ductus arteriosus is closed or very narrow, the bronchial arteries become {706} the means of supply for the lungs, and through them the blood passes to be aërated. Branches from the coronary arteries have been found supplying a partial channel for the blood to the lungs, as well as the oesophageal, pericardial, internal mammary, and intercostal arteries.

The duration of life is often very considerable. Thirty-seven years have been attained.

4. Combined Stenosis and Atresia of the Pulmonary Artery.--Under this division are arranged other primary defects of the heart, which are found combined with stenosis and atresia of the pulmonary artery. It is very striking how frequently this artery is narrowed or closed in defects of the heart which date from the early period of foetal life, before the division of the truncus art. comm. and of the ventricles has occurred. It is only very rarely that defects from this early period show a normal width in this vessel; in the great majority it is narrowed or closed. The aorta is rarely affected in this manner. Changes in the aorta may come also, but a complete failure or great narrowness of this circulation is so difficult to overcome by a collateral circulation--more difficult than the pulmonary circulation--that life must cease in the foetus, or at least the conditions are incompatible with extra-uterine existence.

A. Combination with partial persistence of the truncus arteriosus communis. The defects coming under this head show usually very great deficiency of the organ and its great vessels, although the heart itself in rare instances shows the proper arrangement of the cavities and their valves. The persistence of the truncus art. comm. may be complete or partial; the defect consists in the total absence or arrest of growth of the septum of the truncus, which partitions it into two portions. Normally, the two septa grow simultaneously and meet at the base of the heart. In cases of persistence of the truncus art. comm. the upper septum fails to develop. In incomplete division of the truncus the pulmonary artery suffers more than the aorta, and the former is always narrower than its fellow-vessel. This difference varies greatly. The valves of the pulmonary artery often fail entirely, and the ductus art. Botalli is many times absent.

B. Combination with cor biloculare.--Here we have a heart consisting of two cavities--one auricle and one ventricle--where no partitioning has taken effect. The defect results from the failure of the septum ventriculorum to grow; and with this, as in the former division, comes also a more or less complete failure of the septum trunci art. comm.

C. Combination with single ventricle and divided auricles (cor triloculare biatriatum).--In the cases of single ventricle with more or less complete division of the auricles the pulmonary artery generally shows narrowing to a greater or less degree; it may still be pervious, although its orifice is closed, or it may be throughout entirely obliterated. The valves may be entirely wanting. The duration of life is very short, though in a very few with effective compensatory changes it may be prolonged very considerably.

D. Combination with divided ventricle and a single auricle (cor triloculare biventriculare).--In strictness, this defect is nothing more than an open foramen ovale with some deficiency of the pulmonary artery; but, in reality, the heart is much more malformed. The whole septum atriorum is wanting; the superior or descending vena cava is doubled--one entering the left part of the common auricle, the other opening more to the right. The ventricular septum shows a greater or less defection, the pulmonary artery is narrowed, and the aorta arises from both ventricles or wholly from the right one.

E. Combination with special anomalies in the position of both the great arterial trunks.--Here come a variety of anomalies in the arrangement of the aorta and the pulmonary artery in relation to their respective ventricles and to themselves.

{707} a. In transposition of the great arteries, the aorta arising from the right ventricle and the pulmonary artery from the left cavity, either there comes a general transposition of all the viscera or the heart alone is reversed. Very rarely in transposition of the vessels the septum ventriculorum is closed, commonly open, and although the size of the vessels may be normal, usually their relation and position continue reversed throughout their course. In cases where the pulmonary artery is narrowed the duration of life is short. b. The pulmonary artery may arise from the left ventricle and the aorta from both ventricles; or, c, the aorta may come wholly from the right ventricle, and the pulmonary artery from both cavities; the latter vessel may be narrowed or show its normal width or even be considerably dilated. d. Both the great vessels may arise from the left ventricle, very much dilated, with the aorta in front of the pulmonary artery and the latter narrowed. e. The relation of the great arteries may be found reversed--i.e. the aorta in front and the pulmonary artery behind, and the aorta spring from both ventricles and the pulmonary from the right alone.

F. Combination with primary defects of other valvular orifices of the heart.

a. The tricuspid valve may be quite rudimentary, producing by the regurgitation thus allowed, especially when combined with pulmonary stenosis, great dilatation of the right auricle. When the pulmonary artery is narrowed the septum ventriculorum remains open; the aorta carries the blood, distributing it to the lungs by an open ductus arteriosus Botalli or a collateral circulation. The collateral circulation is less developed the greater the width of the pulmonary artery. The foramen ovale may close in such a case, but when it remains open the relief to the over-distended right auricle is very great.

b. Many cases of congenital stenosis and atresia of the right auriculo-ventricular orifice are reported in which the condition of the pulmonary artery is not described. In fact, it is a difficult matter to determine if the auriculo-ventricular narrowing is a primary one. Its defective size may be merely, as it were, a rudimentary condition, a failure to enlarge through disuse. When the pulmonary orifice is closed and the right ventricular cavity remains small, the tricuspid orifice is naturally small in size. There are, however, undoubted cases of tricuspid narrowing with or without stenosis of the pulmonary artery; the defect consists in a primary contraction of the fibrous ring or in the union by partial adhesions of the leaflets.

Malformations affecting primarily the Left Side of the Heart.

Primary defects of the systemic side of the heart are, for the reasons already given, very much more rarely seen than those of the pulmonary heart. In such cases the aortic conus and its orifice are found more frequently affected than the mitral orifice; both of these orifices, however, may be congenitally altered without foetal malformations at other parts of the heart being present; such cases are on record, though only sparsely scattered through the literature of cardiac diseases. Dilg[8] has recently made an important addition to this subject. He proposes a classification on a new basis for all forms of cardiac malformation; to these classes he makes conform the malformations of the left side of the heart. In the first class he places all cases dependent on an inflammatory process occurring in the foetal heart after its normal development is completed; in the second, those cases of malformation in which the deviation from the normal consists in defects of formation; in the third, those which present a combination of endo-myocarditis with defective development.

[Footnote 8: Virch. Arch., Bd. xci., S. 193-259, 1883: "Ein Beitrag zur Kenntniss seltener Herzanomalien in Anschluss an einem Fall von angeborner linksseitiger Conusstenose."]

{708} Among the many cases of malformation of the heart which he presents there are reports of 15 cases of stenosis of the conus, which are to be divided into two categories, in accordance with his classification. In the first group, in 7 cases the stenosis is due to an inflammatory process, and is conditioned by the results of the endo-myocarditis localized in the aortic conus. These cases must have originated at a late period of foetal life, and they correspond closely to the conditions arising in the adult organs from similar processes. In all the specimens the mitral orifice was involved, and contributed a share in the production of the conus stenosis; in all the cases the aortic valves also had suffered inflammatory changes. Here Dilg also speaks of a band-like hypertrophy of muscular fibres, marking the outlines of the aortic conus, similar to the condition described by Peacock in the right ventricle; in this condition there was no evidence of endocarditis, and the condition may have been due to cadaveric rigidity. The left ventricle presented varying conditions according to the state of the aortic and mitral valves; in some cases there was concentric hypertrophy, or, more strictly speaking, narrowing or shrivelling of the cavity with hypertrophied walls; in others dilatation existed. The other cavities of the heart were influenced by the competency of the mitral orifice, but almost always showed considerable hypertrophy and dilatation. The valvular apparatus of the right heart was not free from evidences of old inflammation, but this condition was not very marked. The ages of the reported cases reached from thirty to seventy-five years.

In the second group there are eight cases in which the defective condition of the aortic conus caused malformation of other parts of the heart. Here the conus stenosis occurred at an early period of foetal development, before the permanent structures of the heart were fully formed. The conus stenosis is to be considered as primary, the other defects as secondary. As we have already seen, the left side of the heart is much less liable to deforming causes, and when such do occur the secondary defects are less conspicuous. Thus, in only four of these cases were there such malformations of other parts of the heart as openings in the septa of the ventricles or auricles, patency of the ductus arteriosus Botalli. The defects consist more usually in what were formerly called excesses of development, such as the formation of bands below the aortic orifice; or of deficiencies of development, such as only two aortic valves. These conditions are very doubtfully due to formative excesses or deficiencies, but rather to intense inflammatory processes or other morbid conditions which have resulted in the formation of excessive cicatrices or the removal of normal parts.

Another division of cases shows narrowing of the aortic trunk itself. This condition is probably always a true defect of development; so far as these cases, collected by Dilg, show, it is unquestionably so. In narrowing or closure of the pulmonary artery trunk it is found that in some instances this condition was dependent on an endarteritis resulting in a partial occlusion of the lumen of the vessel; here, however, the aortic trunk furnishes no evidences of such a process. It must therefore be due to an unequal division of the truncus arteriosus communis. The cause and the mechanism of this unequal division of the common trunk, resulting in a reduction of the size of the aorta, are probably similar to what Rokitansky indicated for the reduction in the size of the pulmonary artery trunk.

In the specimens of aortic narrowing (no cases of complete closure are reported) from this cause and mechanism the pulmonary artery has been found unusually wide, but this condition of the pulmonary trunk is not very conspicuous, and does not necessarily result from the narrowed state of the aorta. The compensatory or secondary defect of open septum ventriculorum, or even of the auricular septum, is, in these cases, neither invariable nor necessary to a proper maintenance of the foetal or adult circulation. In fact, {709} the open ventricular septum is rare; the condition of the auricular septum is, in the reports, often not stated. The left ventricular walls commonly show a preponderance of hypertrophy over dilatation of this cavity, but in some cases the distension of the cavity is marked.

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SYMPTOMS.--The most striking symptom which occurs in malformation of the heart is the cyanosis, but the appearance of this peculiar symptom may be postponed until some time, even a long period, after birth. In the newly-born infant presenting a blue color the diagnosis rests between the not infrequent temporary failure of respiration from many causes and a defective development of the circulatory organs. In most cases the doubt is promptly solved by the voluntary or artificial efforts of breathing, whereby the cyanosis disappears. If the dark hue persists after the respiratory movements have been developed, the cyanosis may be found to depend either on cardiac malformation or an imperfect expansion of the lungs (atelectasis). The distinction between these two conditions can usually be made by a study of the respiratory movements, by the state of the heart's action and of the pulse, aided sometimes by an inspection of the outlines of the chest. In cardiac malformation respiration seems to be well performed and full, though often hurried or labored; in atelectasis this function is often found characteristically altered by being short, high, and imperfect, with imperfect distension; the ribs, instead of moving upward and outward, fall toward the median line, and the chest fails to expand transversely. In malformation the heart's action and the pulse are rapid, and a murmur can often be heard. The thoracic outline may deviate from the usual antero-posterior flattening by the sternum being prominent in cases where the heart, instead of its usual position to the left, is placed more centrally, as comes in certain defects of development. Both of these conditions may be present, and then the symptoms are mixed in character.

Cases of atelectasis, sufficiently marked to give rise to persistent cyanosis, if not relieved too frequently show a pretty rapid increase of color, becoming deeply livid, with convulsive movement, ending shortly in death. The diagnosis in such cases between a cardiac malformation and a non-expanded lung is almost impossible unless the respiration shows characteristic features. It is probable that the treatment proper for the latter would aggravate the condition of the circulation in malformation. In a majority of cases a post-mortem examination is necessary to determine whether the cyanosis is of cardiac or of pulmonary origin. In the atelectatic condition, if death comes within a few days of birth, the ductus arteriosus Botalli and the foramen ovale may both be found open, especially the latter, their time of normal closure not having arrived; in cases dying at a later period, if the foetal openings are still found patulous, the open state must be considered as dependent on the condition of the lung-tissue, since in malformation of the heart the patulous state of these foetal openings is, as has already been shown, rare as a primary defect, and, except in connection with defects of development resulting in obstruction, which operate at other points of the foetal circulation, is almost never found. In other words, an open foramen or ductus is a secondary defect, dependent, on the one hand, on a primary obstruction of the cardiac ostia, or, on the other hand, it may be on a primary atelectasis or malformation of the lungs.

If the child passes beyond the first weeks of life without exhibiting cyanosis, the subsequent occurrence of the condition becomes almost a pathognomonic symptom of cardiac or vascular malformation, unless it can be shown that the coloration is dependent on some acute disease, especially acquired valvular disease: in this connection collapse of the lung (post-natal atelectasis), too, must be remembered.

{710} It is during the first week of life that cyanosis makes its appearance in the great majority of cases of malformation of the heart, in the proportion of more than two to one of the cases. The coloration, once developed, may remain permanent and of equal intensity until death, but as less than 8 per cent. of infants with malformation die within the first week, and only 36 per cent. within the first year, this symptom usually remits. It may wholly disappear, to return on very slight provocation, such as excitement, or on exertion, on the advent of acute disease, or without apparent cause. Probably about one-fourth of those who die in infancy perish in paroxysms of dyspnoea, another quarter of acute disease, and the remaining half of convulsions; and toward death the cyanosis generally becomes very intense.

If the malformation is not of character or degree to develop cyanosis early in life, the child grows and passes through the usual stage of development, usually, however, feeble, poorly nourished, incapable of common exertion, but often without any special phenomena to attract attention, and the vice of formation is undetected unless by a special examination. There are several other symptoms frequently present in connection with malformation, but not of a pathognomonic character. Dyspnoea, though rarely occurring without cyanosis, may attract attention, and, if frequently brought on by active exercise, increases in violence, to be later accompanied with the cyanosis originally absent. Palpitation is not uncommon, especially in cases of great hypertrophy with dilatation, in hearts struggling to overcome an obstruction; in other cases it is absent or only occurs on exertion in connection with dyspnoea and cyanosis. The degree of animal heat varies greatly, judging by the various opinions expressed by writers. The sensation of patients able to express their feeling is often that of chilliness, and in some cases the surfaces of the body feel cold, although the indications of the thermometer show no great variation from the normal temperature. It is obvious that no very great variation from this standard is compatible with the long duration of life, although a depression may exist during or immediately after paroxysms of dyspnoea or cyanosis. Cough is also frequent, but is probably always due to some acquired pulmonary disease.

The physical signs offer increased facilities for the recognition of defects of development. In the early reported cases there are of course no records of these conditions, and there is therefore a lessened number of instances from which to collate the physical signs. In the early days of life it has been shown wherein the presence of a cardiac murmur may lead to the distinction between malformation and atelectasis. In later periods of life the physical signs cannot be regarded as characteristic. There are no signs by which a malformation can be distinguished accurately from an acquired cardiac disease, so that without the clinical history and a grouping of symptoms the diagnosis cannot be made from the physical examination.

Inspection and palpation of the chest often show the heart to be in an unusual position, placed more centrally under the sternum. It must be remembered that transposition of the heart to the right side is not unfrequently unaccompanied with any malformation of its ostia giving rise to symptoms; and this organ may be even more markedly displaced without being malformed, although under both these conditions irregularities of the principal trunks are usually found.

Percussion frequently shows enlargement of the area of cardiac dulness, but, on the other hand, at the post-mortem examination the heart is often found markedly defective without externally showing variation of its size or shape, or of its position within the thorax; hence in such cases no deviation from the normal will be revealed on percussion or inspection of the chest. It is probable that cardiac murmurs are not always to be detected in cases of even marked defects of development, but when present it is recorded most {711} frequently that a single murmur is heard over the base of the heart, blowing in character and systolic in time. Such a sound is probably produced by the passage of the blood through an abnormal opening between the ventricles or through the foramen ovale. Other murmurs may also be present; if the arterial ostia are defective from narrowing, roughness, or insufficiency of their valvular apparatus, abnormal sounds of different characters, diastolic or systolic in time, may be heard. Too few observations as yet exist for a general diagnostic scheme to be formulated. Auscultation of the intra-uterine heart may in the future become sufficiently accurate to enable us to prognosticate a congenital cardiac malformation or disease; there is one case on record in which a correct diagnosis was made in this way.

The ends of the fingers and toes are frequently described as bulbous. This rounding and retraction of the nails, frequently spoken of as clubbing, does undoubtedly exist in many cases, but the condition cannot be regarded as characteristic of malformation of the heart, since it comes with even more frequency in tubercular disease of the lungs, in chronic pleurisy, and in other chronic pulmonary maladies.

Lebert has recently insisted on the connection between stenosis of the pulmonary artery and tuberculosis, not merely as a coincidence, but as the cause of the development of the tubercles in the lungs. Many others have spoken of this connection, and very many are the cases recorded--perhaps nearly one-quarter of the whole number. In some cases large or small single cheesy masses exist; in others cavities form, and in rare cases a miliary tuberculosis exists, still more rarely affecting other organs than the lungs. In view of the recent dogmas of tuberculosis it is doubtful if many of these authors would at present insist on the connection between malformation of the heart and tuberculosis being other than a coincidence, since it is not apparent why such patients are more likely to be invaded by a bacillus of tuberculosis than other persons, and this organism is known to grow so readily wherever the spores chance to fall.

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DURATION OF LIFE.--In connection with certain malformations some indications have already been given in respect to the duration of life in such defects. It is, however, apparent that the degree of the obstruction to an orifice or vessel, and still more the completeness of the secondary compensatory alterations, exert a greater influence than the seat of the malformation on the continuance of life. The occasional slight isolated malformations, such as open septa without obstruction of the orifices, in themselves often entail no symptoms, and, unless combined with acquired valvular disease, exercise no influence on the duration of life; here, however, the prognosis merges entirely into the acquired malady.

Of the other conditions of malformation, narrowing of the aorta and of the aortic conus seems to be, on the whole, compatible with a longer duration of life than any other condition, and these defects cause death in the early days or months in fewer cases than similar obstructions on the right side of the heart. This result apparently comes from the fact that the left ventricle seems to possess unlimited capacity for hypertrophy, and hence is able to overcome the obstruction; when the aortic valves allow of regurgitation the compensation fails and death comes sooner. When the main branches of the aorta are defective or when the descending aorta is derived from the pulmonary artery, the duration of life is much shortened.

In cases of pulmonary narrowing in general it may be stated that the greater the obstruction the shorter the life. This rule is subject to many exceptions; so frequent are the exceptions that the rule is almost valueless for determining the life in any given case. Complete closure of the pulmonary trunk has permitted of the continuance of life for sixteen years, {712} and then ended from an intercurrent acute disease. When the septa are maintained open--when, therefore, the communications between the pulmonic and systemic sides of the heart are free--a greater age is attained than when these openings have become closed. This condition of the pulmonary artery in order to permit of a long duration of life must be coincident with a considerable development of the collateral circulation by which the blood freely enters the lungs for aëration; otherwise the compensation fails very soon.

In transposition of the main trunk relatively to the ventricles, with closure of the septum ventriculorum (very rare), life ends not many weeks after birth; if the septa remain open, which is not common, life may be prolonged for a year or two.

Cyanosis.

There are two views to be found, set in opposition to each other, to account for the peculiar blue coloration of the skin and mucous membranes in cases of malformation of the heart. The first explanation attributes the phenomenon to a general congestion of the venous system, due to the obstruction of the pulmonary artery. This view was proposed by Morgagni in connection with his, the first described, case of malformation of the heart. The other view considers that the intermingling of venous and arterial blood through any channel, but especially by means of abnormal openings in the septa, produces the blue coloration. Numerous writers have defended each of these theories of causation; from most of their observations darkness rather than light has resulted through the attempt to defend one or the other theory exclusively.

Gintrac defended the admixture theory for cyanosis, and his views became so well known that a large majority of persons conformed their belief to his teachings. This author distinguished four varieties of blue coloration: first, that due to some malformation of the heart or great vessels, by which the blood of the right side of the heart enters the systemic arterial circulation; second, likewise due to intermixture of the blood, but produced by conditions developed after birth through the re-establishment of the passages of communication or other changes in the circulation; third, where the coloration appears without direct admixture of the blood, but from organic disease of the heart; fourth, cases without malformation, from a suppression of the menses. Before the time of Gintrac, cyanosis had a very indefinite signification, and the condition was looked upon, and was classed by very many, as one of the cachexiæ, and was often spoken of as a form of icterus. He, however, held that the organic lesions of the heart and great vessels were the necessary conditions of its production, and that the mixture of the red and black blood, and the distribution of the mixed fluid by means of the arteries to all parts of the body, determined its essential character. He showed, too, that all communications between the right and left heart were not followed by cyanosis; the explanation of the absence of the blue color was that from the simultaneous contraction of the auricles and ventricles of the two sides of the heart an equilibrium was produced, and the blood did not deviate from its normal course. This result followed only when the normal exits of the blood were unobstructed. This supposition, as is apparent, is not in accord with the facts. During the filling of the ventricles, before the muscular contraction of the walls occurs, the blood has the opportunity of freely mingling if the opening between the cavities is sufficiently large: that the blood will not thus mingle when the muscular contraction acts remains to be proved. Cases of open septum ventriculorum, as an isolated defect, without obstruction of the great vascular trunks (a rare condition), are not attended with cyanosis: the absence of this symptom, as will be shown later, is readily to {713} be explained on other grounds than those supposed by Gintrac. The normal outlets of the blood are, however, almost always obstructed to a greater or less degree; and here the explanation of the absence of the cyanosis fails. In the delayed appearance of cyanosis Gintrac considered the reason to be that the venous blood differed less from the arterial in the young subject than in those of more advanced age, because, on the one hand, the aëration was more active, and, on the other hand, the deterioration of arterial blood was less marked. In other cases he points to an increase of the obstruction, through inflammatory changes, as the probable reason for the delayed appearance of the blue color; in still other cases it was supposed to be due to a disturbance of the equilibrium of the pulmonary and systemic circulation from an increase in the blood-mass. In cases of unilocular and bilocular hearts, of which the author speaks, his explanation completely fails, for here the admixture of the blood within the heart is very marked; yet such cases have been reported without cyanosis. He further believed that openings in the ventricular septum, as well as between the auricles, were effected after birth as the results of acquired cardiac disease.

Gintrac, in speaking of the causes of cyanosis, says that the condition shows no hereditary tendency; that the pregnancy during which the defective infant is developed is without noticeable phenomena; and that the confinement is normal. It is on some of these points that we are in want of accurate information. It has been pointed out that many congenital defects of the heart result from morbid processes affecting the organ during its developmental stage. These lesions are the same in kind as those which produce cardiac and vascular disease in the adult, and are likewise of a sort capable of communication from the parent to the foetus. Such diseases are found acting oftentimes temporarily in the parent; and if they acted during pregnancy, or even if present only at the time of conception, their results would rationally be expected to be displayed in the foetus. Such diseases as rheumatism and syphilis, which may be regarded as temporarily-acting maladies, would come under this class, and doubtless many others might be added to the list. The work of collecting the histories of pregnancies or the condition of the parents at or before the time of conception would be painfully tedious: such records do not exist at present, and they could be made sufficiently full only in exceptional cases; but their value in determining the causes which operate in the production of defective development of the heart cannot be too highly estimated.

The conclusions stated by Moreton Stillé[9] seem to be the first which justly cover the ground from a comparison of large numbers of cases of malformation of the heart. The first conclusion by him is that cyanosis may exist without admixture of the blood; by this was meant that no abnormal communication between the right and left sides of the heart, and no channels between the principal vascular trunks, are present. He mentions five cases of cyanosis occurring in which no means of admixture existed. The second conclusion is that there exists no proportion between cyanosis and the degree in which the blood is mixed; for this he cites four cases, some with the aorta arising from the right ventricle, others of hearts with only two cavities and the common trunk undivided, in which the cyanosis was only partial or transient. The third conclusion, the converse of the first, and reinforcing the preceding one, is that complete admixture of the blood may take place without cyanosis. The fourth, that the variation in the extent, depth, and duration of the discoloration is inexplicable by the doctrine of the mixture of the blood.

[Footnote 9: "Inaug. Thesis." Amer. Journ. Med. Sci., N. S., vol. viii., 1844.]

Having shown that commingling of arterial and venous blood cannot be the cause in itself of cyanosis, Stillé proceeds to the study of the other {714} theory--viz. that it is due to congestion of the general venous system resulting from some obstruction in the right side of the heart or in the pulmonary artery, impeding the passage of the blood through the heart. These structural lesions must fulfil the three following indications: 1st, that they shall be sufficient in degree to account for the symptom; 2d, that they be present in every case of cyanosis, or in their place some other cause acting on similar principles; 3d, that they shall never exist without cyanosis or without a satisfactory explanation of the exceptional occurrence. He holds that contraction of the pulmonary artery is to be taken as the type of all the lesions that may produce cyanosis, and that this type fulfils the indications given above.

Most writers since Stillé have coincided with him, or have regarded cyanosis as partly due to venous congestion and partly to commingling of arterial and venous blood. Some writers, however, have pointed to the abnormal communications between the right and left side of the heart, and asked why, if admixture of the venous and arterial blood is not the cause of cyanosis, should the admixture through such openings be found in such a large proportion of cases. Such writers have failed to distinguish between the primary and secondary defects of development. They have failed to see that the pulmonary obstruction which prevents the blood during extra-uterine life from passing to the lungs for aëration, and consequently produced the cyanosis, prevented the closure of the ventricular septum during intra-uterine life, or of the auricular septum within a few days of birth. In reply to the above question it may be pointed out, as Peacock has done, that such communications between the two sides of the heart are all important for the continuance of life, even for the shortest period, when the pulmonary artery is occluded.

It is evident, as Peacock has shown, that if Stillé's first and third conclusions are true, as the cases undoubtedly show, the theory of intermixture of the blood does not account for the condition of cyanosis. It is probable in many of these cases with abnormal openings in the septa that the intermixture of the blood is but slight, since if the pressure on the two sides of the heart is equal--and it may become equal through the establishment of a collateral circulation, although primarily it was unequal--no intermixture takes place through the defective septa. Neither does the admixture theory account for cases of intermittent or delayed cyanosis. Such cases can only be supposed to be due to a varying propulsive power or to a subsequent increase of the pulmonary obstruction. Neither does admixture account for localized cyanosis; for example, in the face or in one extremity: this condition, rare as it is, must be due to other causes. Peacock, while combating the admixture theory, considers that Stillé's conclusions in favor of the congestion theory as dependent on obstruction of the pulmonary artery are too exclusive. He discusses also the relationship of congenital cyanosis due to malformation, to cyanosis acquired through pulmonary and cardiac disease as seen in the adult, and shows why the latter condition is rarely ever as intense as the former, and also why acquired obstruction of the pulmonary artery is not necessarily productive of cyanosis. The reason of the difference he believes consists in the compensatory hypertrophy of the right ventricle, with perhaps a gradual diminution of the blood-mass, as seen in some cases.

On the whole, Peacock subscribes to the congestive theory, but thinks that the intensity of the cyanosis is modified by the capacity of the capillaries, by the period of development or duration of the obstruction, by the natural coloration of the skin, and by the color of the blood itself.

Under these two theories, and the arguments offered in support of them, there seems to be no other explanation possible of the condition of blueness, and yet the whole story of the mechanism of cyanosis does not seem clear. Partly, this is due to the incomplete knowledge of the physiology of the aëration of the blood which obtained during the most active period of the {715} discussion of cyanosis and its causation. Let us consider briefly the simplest case of cyanosis. Every child born has in one sense a temporary malformation of the heart--an open foramen ovale which does not close for several days after birth. Every child is born partially cyanotic, owing to compression of the uterine sinuses or pressure on the umbilical cord; it is completely cyanotic if there occurs premature separation of the placenta. The cyanosis continues until the child breathes. The cause of this cyanosis must be looked for, not in the temporary malformation, but in the imperfect expansion of the lungs. As soon as the respiratory function is assumed--as soon as, in other words, the pulmonary-artery branches carry a full amount of blood which becomes aërated in the lungs--the cyanosis ceases, although the foramen ovale is not yet closed.

The closure of the foramen by a trapdoor valve is, as has already been pointed out, not in accordance with the anatomical facts: turning the newly-born infant on its right side does not favor, as it is commonly supposed, the closure by gravity of a preformed swinging lid, which when it has dropped down for ever partitions the right from the left auricle. The right-sided position may favor the expansion of lungs or in other ways promote the pulmonary circulation, but in itself it does not tend to close the foramen. In fact, cyanosis does not here depend on the defective development, but on want of aëration of the blood.

Again, looking to the skin or mucous membrane, what is the condition of the blood and of the circulation which renders the parts of a blue color, and in what do they differ from the normal? In the normal state of the blood and circulation the capillaries of a given area are filled, one half with arterial blood, and the other half with venous blood; that is to say, the capillaries at the point of their origin from the arterioles contain pure arterial blood: as the blood-current proceeds outward the blood becomes progressively less and less red and more and more blue or black; when the venous radicle is reached the blood-current is of as dark a hue as it ever becomes. In general terms, therefore, it may be said, taking the average, that in a given area half the blood is venous, half arterial. Here, then, we see, with an equal mixture of the red and blue blood, nothing resembling cyanosis. It is evident, therefore, that to produce a cyanotic hue the blood must be wholly venous; the intensity of the blueness will vary with the amount of non-aërated blood present in the capillaries. But let us suppose an equal admixture of right- and left-sided blood to take place--for example, when the aorta arises from both ventricles, the pulmonary artery obstructed. It cannot be supposed that the venous blood would retain its dark hue. The contact of the two bloods within the aorta on their way to the capillaries would result in arterializing the venous blood at least one-half, so that when it arrives at the capillary network the intensely blue color of a marked case of cyanosis would have disappeared.

Besides this, there are other considerations to be taken into account to show that neither of the two exclusive theories accounts for the state of the blood and of the circulation in cyanosis. If the condition of the cyanotic parts, due to acquired valvular heart disease or various morbid states of the pulmonary tissue of an acute character be compared with the same parts in cyanosis from malformation of the heart, striking differences are discernible. If the simple condition of cyanosis of the part due to localized pressure on the veins be examined, the differences are even more perceptible. In the malformation there is an admixture of blood; in the other condition there is no opportunity for the intermingling of the currents. In the latter the cyanotic area becomes swollen, and the intensity of the color may become lessened through the oedematous condition; in the former the skin of the cyanotic infant rarely if ever presents any swelling; the veins of the part show little, {716} if any distension, as is so frequent in the latter; cases of malformation in which subsequent endocarditis with additional obstruction occurs may show oedema and swelling similar to cases of acquired valvular disease. In these cases of cyanosis the condition must be due to a want of aëration of the blood, since it never appears until such alterations of the pulmonary tissue and circulation are reached as to render it certain that the blue coloration is due to a want of aëration of the blood. Fulness of the veins and oedema may be present, but never general cyanosis.

Another important consideration in the production of cyanosis does not seem to have been fully appreciated. It is the fact that in all cases of obstruction of the pulmonary artery the collateral circulation, carried on by very varying channels, the bronchial arteries, the oesophageals, the coronaries in some cases, the internal mammaries and intercostal arteries in rare cases, or by the ductus arteriosus Botalli, which alone must be always inadequate in marked narrowing of the pulmonary trunk,--the collateral circulation must always remain insufficient for carrying sufficient blood to the lungs for aëration. Kussmaul was the first to call particular attention to this fact; and it is to this condition of insufficient channels for the blood reaching the lungs that certain cases of cyanosis must owe their causation.

Hence it must be that, in all the complex conditions found in cases of cyanosis from defective development of the heart, a want of due arterialization or aëration of the blood is at the foundation of the state as seen in the cyanotic area. Whether it results in a given case from excessive admixture of venous blood with the arterial when the current reaches the capillaries, or from venous stasis due to central obstruction, of which pulmonary-artery narrowing or closure is the type, or whether from a failure of sufficient blood to reach the lung, as where the collateral circulation remains imperfect, or as seen in certain cases of defective development of the lungs, is most difficult to ascertain. That sufficient consideration has not been given to the third possible factor in the causation of cyanosis--viz. failure of the blood to reach the lung, as distinguished from general venous congestion alone--is evident. That intermingling of the blood from the two sides of the heart must inevitably reduce the red color is certain--that in very many cases the reduction in color does not cause cyanosis can be readily understood from the consideration already offered. The cases of free admixture in which cyanosis does occur may coincide with a condition of very imperfect collateral circulation to the lungs, and hence with a low aëration of blood of the left ventricle, insufficient, therefore, to bring up the color of the blood from the right side of the heart above the cyanotic point.

Whether non-aëration of the blood from failure to reach the lungs, apart from general venous congestion, is a sufficient explanation of the cyanosis in a large majority of cases or in the whole number, is not apparent from the records of reported cases. Much more accurate post-mortem accounts, made with a view to determine the question, than at present exist will be required. In a number of well-reported cases of defective pulmonary artery with a free admixture of blood the pulmonary collateral circulation is found to be well developed, and no cyanosis had appeared, or had been but trifling and inconstant. In other cases of quite as marked pulmonary obstruction with but slight commingling of the blood through abnormal apertures and but slightly-developed collateral circulation, cyanosis has been found intense and constant. In the two conditions the possibilities for general venous congestion are about the same, though perhaps not equal, while the striking difference, apart from the admixture of the blood-current, consists in the conveniences for the aëration of the blood.

The only variety of malformation of the heart in which intense and constant cyanosis must inevitably be present is that very rare form of {717} transposition of the great trunks, the aorta springing from the right ventricle, the pulmonary artery from the left, with closure of the septum ventriculorum; the pulmonary veins enter the left auricle bearing red blood, and the venæ cavæ the right auricle with blue blood; if the ventricular septum is closed, the aorta necessarily carries blue blood to the systemic circulation, and the pulmonary artery is filled with red, carrying it back to the lungs, whence the fluid has just come. In such relation of the principal trunks, even if the ductus arteriosus Botalli and the foramen ovale remain open, cyanosis is necessarily present. The bulk of the blood in the aorta is blue: the only points in which it comes in contact with red blood are, first, at the foramen ovale: here the intermingling is not sufficient to bring it above the cyanotic color; and, secondly, at the ductus arteriosus, and here the tube is not favorably directed for a copious intermingling of the two bloods, neither can it probably ever be sufficient in itself for this purpose. Hence the aortic blood is almost wholly venous. If these two foetal openings did not persist life could not continue beyond a few hours, or even a few minutes, after birth. In such a case the cyanosis does not depend on general venous congestion, and specimens are reported of this sort in which the great vascular trunks were without obstruction, life having been maintained for a few months; adult existence is probably impossible. If, however, with such transposition of the vessels to the improper ventricle, the septum ventriculorum remains widely open, cyanosis may be absent or inconstant, because, apparently, admixture of the blood and also aëration are sufficiently free. But in cases of transposition of the vessels, or even in the much more frequent specimens without transposition, when the track to the lungs is defective either from want of a collateral pulmonary circulation or directly from impervious pulmonary artery, cyanosis becomes more intense and more constant or comes in more frequent paroxysms, irrespective of the presence or absence of evidences of general venous congestion.

It would seem to result from this grouping of facts, and looking at them from a reverse bearing to Moreton Stillé's point of view, that distal rather than proximal obstruction of the pulmonary artery, taken as a type, was the cause of cyanosis. Admixture of arterial and venous blood must reduce the redness of the arterial stream, just as certainly as red paint mixed with black varnish will render the black less intense: whether admixture alone ever produces a deep cyanotic hue of the surfaces is probably more than doubtful; that admixture will prevent constant cyanosis seems certain, when cases of complete transposition of the vessels with open septum ventriculorum are compared with those with closed septum, the other conditions remaining the same. General venous congestion from pulmonary obstruction or other causes outside the pulmonary tissue produces cyanosis, but of a sort quite unlike the typical cyanotic condition of malformation of the heart. It may therefore be doubted if the cyanosis seen in obstruction of the pulmonary artery is due to general venous congestion; it may be wholly produced by conditions on the other side of the obstruction--viz. want of aëration of the blood, which must ever remain the essential feature of cyanosis. This supposition allows of an easy explanation of the difference between cases of apparently equal obstruction of the artery, in some of which cyanosis is present and in others absent; it also allows of the explanation of inconstant or paroxysmal cyanosis where the obstruction, and consequently the venous congestion, is uniform and permanent.

{718}

CARDIAC THROMBOSIS.

BY BEVERLEY ROBINSON, M.D.

DEFINITION.--In general, this name is given to every deposit of coagulated blood or fibrin in one or more of the cardiac cavities. By its derivation ([Greek: thrombôsis], coagulation, from [Greek: thrombys], clot) it further implies the manner in which the coagulum is formed and all the morbid alterations connected with it.

SYNONYMS.--Heart-clot. Fr. Thrombose cardiaque; Ger. Blutgerinnungen im herzen; Lat. Thrombosis cordis; It. Trombo; Sp. Trumbo.

The definition offered is not wholly satisfactory, because, although it is accurate as far as it goes, it is not complete. It does not distinguish between concretions of different origin, etiology, mode of formation, and age. No separation is made between fibrinous deposits which increase from the beginning and layer by layer in the cavities of the heart, and those transported there from a distance and forming a nucleus for fresh deposits. To make the definition anything like exhaustive would require many references to the general history of THROMBOSIS AND EMBOLISM; we therefore direct our readers to that article for what relates to the common facts of these morbid processes, retaining for the present only those matters which relate specially to the heart.

Heart-clots may be formed--1, during life, when the patient enjoys, apparently, good health and strength; 2, toward the termination of life, when the general forces are evidently depressed, or at the final stage, when life ebbs low and the agony has appeared; 3, after death. These clots have therefore been divided into cadaveric clots, those of the agony, and ancient clots. To the clots of the agony exception is properly taken, for the reason that agony is a term employed with a somewhat badly-defined signification. At what period does it begin? Is it not frequently of different length? Does it always exist? The answers to these different questions render our objection proper, and show that we had better employ the word terminal for coagula of the second division.

Manifestly, the separate varieties of coagula have not an equal importance. The clots which are post-mortem productions are only interesting for their physical characters, which, fortunately, are well marked, and enable us at once to distinguish them from the two other varieties. The coagula in the first two divisions have an interest both clinical and pathological.

The ancient clots are invariably accompanied with signs and symptoms which should reveal their presence. As much might be affirmed for the terminal clots in the majority of instances and when the patient is not already in extremis. The pathological study of these varieties has great value, and especially in so far as it will the better enable us to distinguish the clots formed some time previous to death--be it of shorter or longer duration--from those which are but the result of the gradual stagnation of the blood-current in a weakened and wellnigh powerless organ. Amongst the clots which are formed in the venous system, some are transported, and pass {719} immediately through the heart, to be arrested finally in some of the larger or smaller arterial trunks, whilst others remain in situ in the heart, and are constantly increased by successive additions or layers of new fibrin or cruor.

The nomenclature to be desired is one which would assign different distinguishing names to each variety of coagulum, so that at once its origin, mode of formation, and perhaps too its age, should be exactly determined. The ancients employed the term polyp for deposits of every description in the heart, but such use of the word was, generally speaking, erroneous, since the true polyp is a very rare disease of the cardiac cavities. Bartholetti and Pissini first made use of it, and considered without doubt that the false polyps or fibrinous deposits in the heart were of analogous nature with the true polypi which are found so frequently in the uterus and nasal cavities. No doubt (as has been inferred) the term polyp in regard to these formations came into habitual use owing to lack of familiarity on the part of the older writers with the varied aspects of clots, as well as their ignorance of the distinct appearance offered by sections from them under lenses of great power. This mistake, therefore, is to-day not to be wondered at, if we duly consider how imperfect and unusual in olden times were pathological researches. Heart-clot was, as will be seen in the historical sketch which follows, the subject of numerous prolonged and animated discussions. As a result of these latter, it was ultimately believed that the great differences of appearance and formation which exist between coagula depend in great measure upon their relative age, and it is for this reason that the basis of distinction between their varieties rests mainly upon the period of time previous to a death at which they are formed. When we speak, however, of polypiform concretion or deposit, we approximate nearer the truth and indicate in a measure the local origin of a coagulum. Many others have employed the terms post-mortem and ante-mortem as being the only suitable terms with which to make a distinction between the coagula formed during active existence and those which are revealed only with the scalpel in the dead-house. In the consideration of this subject the symptoms shall be fully described which indicate the presence of heart-clot found during life, whilst in regard to clots formed in extremis or after death it is desirable particularly to show the pathological characters which shall definitely place them. For all that pertains to embolism of the heart we shall refer the reader, except when it is essential to mention certain details, to other articles in this work. Certain authors have erroneously, it is believed, regarded this subject of heart-clot as one of mere pathological interest, stating that the dead-house is the only place to study its origin and many of its organic effects. This opinion should be combated with vigor. Such a view is far too restricted, and it is here believed that the clinical aspects of cardiac thrombosis are worthy of attentive study, and that something better and further should be attained than merely to watch the downward course of a patient thus affected, and to bear in mind the pathological sequelæ of this disease.

HISTORICAL SKETCH.--The questions which have a present interest in regard to heart-clot are very different from those which formerly engaged medical attention. No longer are we uncertain as to the formation of these coagula during life, nor doubtful as to the various and important effects produced by their transport in different organs through the arterial and venous vessels. Thanks especially to the inaugural thesis of Legroux (1827), to those of Le Marchand and Ball (1862), to that of Bucquoy (1863), but particularly to the experimental researches of Virchow (1846-56) and to the observations of Senhouse Kirkes[1] in regard to the formation and transport of emboli into the cerebral vessels, these facts are all matters of ordinary information. There is little doubt that Galen had noted the formation of {720} intra-cardiac thrombi during life, and attributed to them interference with circulation and respiration, and, at times, sudden death. With the exception of Salius, mentioned by Morgagni as having remarked oedema due to this cause, we reach the sixteenth century before again meeting with any detailed mention of a similar pathological condition. Helidé of Padua, according to some,[2] Benivenius, according to others,[3] were the first authors to give full descriptions of cardiac polyps. This, indeed, was the term affixed for a long period to fibrinous concretions in the heart, beginning with Sebastian Pissini (Milan, 1654), who first employed it. The name took origin, without doubt, on account of their resemblance to polyps of the nasal fossæ, and perhaps to the animal thus named. It was particularly at this period that they acquired their significance, and became the subject of animated discussions between distinguished physicians of the last two centuries. Some, exaggerating their importance, attributed to them the gravest and most important symptoms, although a chronic affection of the heart or lungs present at the time was frequently sufficient to explain them; others, like Kerkring (1670) and Jos. Pasta[4] (1737), who contested the possibility of the blood coagulating during life, and believed they were invariably cadaveric formations, took from them even a pathological interest. This latter extreme opposition to reality originated very soon a mixed conviction, which was that held by Senac and Morgagni. These distinguished observers recognized that intra-cardiac thrombi formed both during life and after death, the former being rarely encountered. The later, or anatomical school, confirmed these views, but also added testimony to show that ancient and terminal concretions were not phenomena of such unusual occurrence as had been previously held. Testa (1810) and Kreyssig (1824) connected fibrinous deposits with inflammation of the heart, and the last-named writer described a disease which he named carditis polyposa. This view and that of Laennec, which attributed globular vegetations to an inflammatory cause, are in our day disproved. Amongst those authors who rendered certain the formation of cardiac clots during life, we should mention a few others whose names have a special importance in this connection as having made a special study of diseases of the central organ of circulation. These are Corvisart, Burns, Andral, and Bouillaud. Since this period the field of research has become far less limited, and investigations have been made in regard to similar coagulations in the large vessels of the body.

[Footnote 1: Med.-Chir. Trans., 1844, pp. 281-325.]

[Footnote 2: Dict. de Méd. pratique, vol. viii. p. 558.]

[Footnote 3: Ziemssen's Cyclopædia, vol. vi. p. 292.]

[Footnote 4: Quoted by Grisolle, Pathologie interne, Paris, 1865, p. 464.]

In 1856 a new era was established in regard to these formations, and especially with reference to their transport. Virchow at this period showed conclusively, after long-continued and accurate clinical observations and experimental researches, that a clot formed on the one hand in one of the large veins might be carried to the pulmonary artery and block up more or less completely the supply of blood to the lungs; on the other, that a portion of a thrombus formed in the left heart-cavity might become detached and plug completely one of the arteries of some far-removed organ, as the spleen or kidney, and thus give rise to those ultimate effects which we now understand under the name of infarction. Thus was first established the new pathological ideas which have become familiar with the words embolus and embolism. True it is that Virchow was not the first writer who had described the facts relating to the translation of portions of coagulum from one region to another of the circulatory system, and its fixation in a particular arterial branch. Already this subject had been clearly and succinctly narrated by Van Swieten. A passage in which the causes and mechanism of apoplexy are referred to gives lucid explanation of this doctrine: "Whatever causes change the blood, lymph, and the matter which supplies the spirits, so as they cannot pass freely through the arteries of the brain, but are there impacted. Such are {721} frequently--polypous concretions in the carotid and vertebral arteries, whether first formed about the heart or within the cranium itself."[5] These ideas of Van Swieten had not, however, produced any very permanent impression, and were almost forgotten, when Legroux (1827) promulgated his view in regard to the possibility of portions of coagulum being carried from the heart into different portions of the arterial system. He published, in fact, two most interesting cases of gangrene of the hand and forearm in which the efficient cause of the disease was found in an obliterating embolus of the brachial artery, which was evidently similar in its nature to the thrombus found in the heart of his patient. It is interesting to remark that Legroux's inaugural thesis, in which these facts were brought to light, was only the forerunner of some very complete articles on the subject of cardiac and vascular concretions, in which he goes over much of the ground which was covered in Germany by the work of Virchow. Legroux published his ulterior researches in the Gazette hébdomadaire, Paris, 1856, pp. 716 et seq. In fact, under the head of correspondence we find in No. 20 of the journal of this year (pp. 349 and 350) an interesting letter from Legroux to the editor, in which he claims for himself the priority of publication (Van Swieten excepted) of the facts pertaining to intra-cardiac thrombi and their effects due to transport of detached fragments into a region more or less removed from their place of development (p. 34). As this claim, according to my researches, appears justified, a part at least of the credit awarded to Kirkes, Virchow, and Schützenberger as discoverers and disseminators properly belongs to Legroux.

[Footnote 5: G. Van Swieten, Commentaries upon the Aphorisms of Boerhaave, Aph. mx., vol. iii. p. 159, ed. London, 1774.]

In spite, however, of these investigations, and those of Allibert (1828), Louis (1837), Baron (1838), and Paget,[6] who showed how the blood could coagulate in the heart and by transport block up the pulmonary capillaries, we cannot dispute the glory to Virchow of having in some sort created this study. Owing to his great sagacity, he was able to seal his studies and experiments with the stamp of a master-mind. The new words embolus and embolism introduced by him refer to a process which was previously but badly understood, and which now fix, as it were, a domain in modern pathology. The theory of Virchow found many advocates--many who were opposed to it in the beginning. In consequence of this it was the origin of numerous works undertaken in this new direction. Amongst the most important are the communication of Schützenberger,[7] the thesis of Lancereaux (1862), the great work of Cohn (Berlin, 1862), the article of Weber in the treatise of Pitta and Billroth--which contains recent theories about coagulation of the blood and the transformation of clots--and the memoir of Polaillon upon cardiac embolism (Paris, 1879).

[Footnote 6: Med.-Chir. Trans., 1844, pp. 162-188.]

[Footnote 7: Gazette médicale de Strasbourg, 1857.]

ETIOLOGY.--So soon as the blood is withdrawn from the influence of life it no longer remains fluid, but rapidly coagulates. Thus it is we find frequently after death coagula filling the cavities of the heart and extending in long ribbon-like bands into the larger vessels, more particularly in the veins. What occurs here is very similar to what we notice in a bowl which receives the blood of a venesection. Here the blood thickens rapidly, the clot forms, leaves the sides of the bowl, assumes the appearance of jelly more or less colored owing to the corpuscles enclosed in the meshes of fibrin, and is bathed in a quantity of ambient serum. A similar change takes place in the heart: the serum is imbibed by the tissues and the clot remains in its cavities.

Coagulation of the blood is a very complex problem. Many theories seek to explain it. On the one hand, it has been said the fibrin pre-exists in the blood, and by the fact of the slowing of the circulation, the reduction of the {722} temperature, etc. the fibrin separates from the blood and coagulates. Again, it is admitted that the fibrin does not exist formed in the blood, but that a fibrinogenous material is present which is acted upon by the hæmoglobin or globulin contained in the red globules, the leucocytes, and the corpuscles of connective tissue, and sometimes is, sometimes is not, caused to precipitate as fibrin (Virchow). The exact conditions which occasion the activity of the globulin are unknown. The reaction which takes place has been said to resemble that which takes place between amygdalin and emulsin when prussic acid is formed, or between myrosin and myronic acid when the volatile oil of mustard is produced. Further, it is stated, in accordance with accurate chemical investigations, that the plasma of the blood contains a substance called plasmin, which separates itself into fibrin which coagulates and into fibrin which remains dissolved in the blood (metalbumen, Robin). These fibrins are evidently of two kinds. The plasmin divides itself under the influence of slowing of the circulation, the action of acids, of foreign bodies, of oxygen in excess, etc.; it remains intact in a fluid condition when the vascular walls and globules are healthy, the blood circulating with normal rapidity, and in presence of alkaline principles.[8] According to Foster,[9] "Coagulation is the result of the interaction of two bodies, paraglobulin and fibrinogen, brought about by the agency of a third body, fibrin ferment." Schmidt concludes that when blood is shed a number of white and intermediate corpuscles fall to pieces, by which act a quantity of fibrin ferment and of paraglobulin is discharged into the plasma. These meeting there with the already present fibrogen give rise to fibrin, and coagulation results.

[Footnote 8: Dict. de Méd. et de Chirurgie pratique, vol. viii. p. 569.]

[Footnote 9: A Textbook of Physiology, p. 22, New York, 1880.]

As regards the formation of clots within the body, it is supposed that injured or diseased spots or foreign bodies first attract, and then, as it were, by irritation cause the death of, a certain number of corpuscles.[10] The views of Schmidt of the fibrino-plastic function of paraglobulin are not accepted by all investigators; and some authors believe that the fibrinogen as well as the fibrin ferment arises from the white corpuscles.[11]

[Footnote 10: Pflüger's Archiv, vi. (1872), p. 413; xi. (1875), pp. 291 and 515; xiii. (1876), pp. 93 and 146; quoted by Foster.]

[Footnote 11: Frédericq, L., Recherches sur la Coagulation du Sang, Bruxelles, 1877, quoted by Foster.]

According to Bristowe,[12] the frequency of sanguineous concretions does not depend upon sex, but is in a certain relation with age. He has remarked, for example, that they are proportionately more often met with at the extremes of life than toward middle age. This might be explained satisfactorily, perhaps, on the supposition that at these periods the circulation is at times very feeble, owing either to congenital feebleness on the one hand or chronic organic affections on the other. At all events, when we seek for the causes which have most influence in determining the formation of cardiac concretions previous to death, we find--I. the mechanical, or those which act specially in slowing the current of blood through the heart. These causes may exist within the heart or may be removed from it. II. The vital or pathological. These causes are of somewhat difficult determination at times, and pertain usually to affections in which there is notable blood-change, in which the quantity of the fibrin has been augmented absolutely or relatively, or to those of infectious type--viz. diphtheria; or to those constitutional in nature--phthisis, cancer, etc. III. The inflammation of the endocardium or endocarditis. This is admitted by Andral, in a note upon the etiology of cardiac concretions in the work of Laennec, as having special importance. Bouillaud also attributed their formation in certain cases to the chemical action of pus which was present in the economy.

[Footnote 12: Pathol. Society's Trans., vol. xiv. p. 71, quoted by Bartholow.]

{723} I. Amongst the mechanical causes we should mention all organic lesions of the heart, all obstacles in the pulmonary circulation, and possibly, by analogy, certain badly-defined lesions of the pneumogastric nerves. All the stenoses and dilatations of orifices, all irregularities of the valves or heart-walls, all depressions or roughened parts of the walls,[13] may determine the beginning of a concretion. In the same way, a small mass of fibrin deposited on a calcareous valve after transport from one of the veins of the limbs may originate a voluminous heart-clot. Dilatation of the heart, pericarditis, every cardiac change which weakens the contractile power, is a predisposing cause of cardiac thrombosis. Every organic lesion of the heart tending toward that final stage of asystolism so often encountered, and which weakens so greatly cardiac contractility; pouching of different portions of the cardiac wall, or aneurism; pressure upon the right heart by a mediastinal tumor or a sacculated aneurism of the arch of the aorta,[14]--all these have great power in producing intra-cardiac thrombi. The mechanism of these different lesions was familiar to Kreyssig, Laennec, and Hope. At the same time it must be admitted that these changes in the heart are not of themselves always sufficient to give rise to fibrinous deposits. We encounter stenoses and regurgitations at orifices very frequently, and concretions, on the other hand, are relatively rare. Moreover, we find heart-clot at times when there is no cardiac alteration. We believe, therefore, that the heart lesion is an aiding factor--that in the last moments of life, when the force of the heart's contraction is weakened and the conditions of the blood favor coagulation, they will act with special power.

[Footnote 13: Pathol. Society's Trans., vol. xiv. p. 71, cases by J. W. Ogle.]

[Footnote 14: Walshe, Dis. of the Heart, Lond., 1873.]

Among the mechanical causes which are removed or distant should be mentioned all those which interfere with the pulmonary circulation. Such are the effects left behind by pneumonia, pleuro-pneumonia, or the compression of the blood-vessels by old congestion of the lungs. In these cases, when the vis a tergo is impaired somewhat, and an obstacle is placed in the pulmonary capillary circulation, even if cardiac thrombosis does not directly result at first, we may have thrombi form in the pulmonary veins. In the same way, the nervous affections which are accompanied with slowing of the circulation tend to produce coagulation of the blood. All lesions, as we have said, of the pneumogastrics act in the same direction. In proof of this we should cite the experiments of Meyer of Bonn, of Longet, and of Blondet, who produced fibrinous concretions in the hearts of animals by tying or cutting the pneumogastric nerves. At the same time, the heart-beats became more rapid, wavering, unequal, and less energetic than in ordinary physiological conditions. After all, however, all these mechanical causes are but predisposing causes, for they do not always produce cardiac concretions. Frequently, as we have said already, the obstruction to the circulation may be present, and yet at the autopsy no fibrinous deposit be found in the heart. In order that the mechanical causes act efficiently to produce coagula, it is essential that they be aided by the conditions of the blood which favor it.

All concretions do not form with the same rapidity nor are they of the same size. At times their production is sudden, and but a few hours elapse before the fatal termination is reached. Again, it is affirmed that weeks, and even months, may pass before the concretion has reached a volume sufficient to cause entire stoppage of the heart's contractions. In the former category are found, of course, the softer, least consistent coagula--usually, however, very voluminous; in the latter are the smaller, more elastic, and resistant concretions, at times even presenting a stratified structure[15] and {724} surrounded habitually by a clot formed during the latter moments of life, and having a large proportion of cruor in its composition. The heart affected with fatty degeneration should, if we consider its weakened power and deficient contractility, be a predisposing cause of stagnation first, and finally of the formation of intra-cardiac thrombi. As a matter of observation in the dead-house, however, such hearts are not frequently accompanied with fibrinous deposits in their cavities.

[Footnote 15: According to Legroux, roughening of the walls or valves gives rise to stratified coagula of moderate size, or else to those small clots which deposit on the surface or margin of the valves (Dict. Encycl. des Sci. méd., article "Concrétions sanguines," Paris, 1876).]

All diseases which by their nature and duration produce great exhaustion of the vital powers tend strongly to produce fibrinous coagula in the heart. This is eminently true of those which at the same time do not occasion a diminished plasticity of the blood. It is often assumed that mere stasis in the blood-current through the heart is essential to the formation of clots in its cavities, and to lend support to this belief reference is made to the phenomena which take place in bleeding. It is not true, however, that stasis is necessary to coagulation, and the proof is afforded when we take a bundle of twigs and by beating the blood forcibly produce the separation of the fibrin. Besides a slowness of the circulation, there must be, once again, an obstacle in the heart itself, and even then polypoid concretions are not always formed.[16]

[Footnote 16: Gaz. hébdomadaire, Paris, 1856.]

II. The Vital or Pathological Causes.--In this class of conditions leading to cardiac thrombosis are included all diseases in which certain special changes have taken place in the blood itself. Among these we should mention, first, certain sthenic inflammatory affections in which the proportion of the plasmin (fibrin and metalbumen) is notably elevated, and in which, on this account, there is a strong tendency to the separation of fibrin from the blood and to the formation of cardiac concretions. In fibrinous pneumonia and acute rheumatism this is particularly true, and amongst the numerous accidents we have to dread in the course of these diseases none strike us with more dread than the possible production of intra-cardiac thrombi. In fibrinous pneumonia this complication is so frequent that Bouillaud has enunciated the following pathological law: "Fibrinous concretions exist constantly in patients who succumb to a frank, acute pleuro-pneumonia, well characterized, which has reached the second stage."[17] According to Raynaud, this is without question a great exaggeration, and results from the confusion this learned author evidently made between terminal clots and those formed some time previous to death. Nevertheless, there is here a proof of the great frequency of coagula occasioned by this disease, and of the strong tendency to their formation which the condition of the blood must afford. What we have said of fibrinous pneumonia and acute articular rheumatism is not true, singular to say, of lobular or broncho-pneumonia. The lesions of this form of pneumonia are those of a catarrhal inflammation of the lung, and the blood does not offer during its course the remarkable tendency to coagulation that is shown in fibrinous pneumonia. Usually, the heart-cavities and the vessels are filled after death with a liquid of a black or violet-brown color, very often sticky.[18] The fibrin in the heart-cavities in pneumonia is fibrillar, and does not present those changes which indicate that it has been deposited for a long while. Moreover, these coagula do not present physical characters which show any considerable degree of age. They are usually terminal coagula, or at least formed within a few days of the fatal termination. Do globular vegetations occur in pneumonia? At times they do, but they are at least very rare as compared with the fibrinous conditions just referred to.

[Footnote 17: Gazette méd., 1843, vol. xi. p. 270, quoted by Armand, Thèse de Paris, 1857, p. 41.]

[Footnote 18: Damaschino, Thèse de Paris.]

There are other general conditions in which there is a marked tendency to {725} the formation of cardiac coagula. In the puerperal state, according to Simpson, it is occasioned by the resorption of many new elements which vitiate its composition and thus occasion this result. In the poisoning following upon glanders or pyohæmia intra-cardiac thrombi are often found. Lancereaux has found in this latter disease fibrinous deposits in the right ventricle and pulmonary artery around small masses composed of pus-cells. In the different cachectic states, such as those caused by chronic Bright's disease,[19] advanced phthisis, and cancer, although we have a diminution in the proportion of red globules, there is present at the same time a relative increase of fibrin; and the consequence is that concretions are often formed in the heart. In fact, it is in these cachexiæ that we often encounter those fibrinous cysts which will be described under the title of Morbid Anatomy.

[Footnote 19: Here the retention of the excreta is an important factor in the formation of cardiac thrombosis (Bristowe).]

Many well-known authors have declared that diphtheria was very powerful in producing fibrinous concretions in the right heart some time previous to death. Among those who have written specially on this subject we would mention Winkler,[20] Richardson,[21] Meigs[22] and Robinson.[23] According to the latter writer, elastic fibrinous clots twisted in the valves and adherent to the cardiac walls are developed frequently in children at a period quite removed from that of the agony, and at a time when they are not as yet in a condition of extreme weakness. Except in exceptional instances this influence of diphtheria to produce cardiac coagula is doubted by Parrot.[24] He admits its power, particularly when it is complicated with membranous croup, and in these examples he believes the precocious formation of coagula is determined probably by the asphyxic condition. Whilst denying the influence of diphtheria, Parrot freely acknowledges that measles, especially when complicated with broncho-pneumonia, tends to produce cardiac concretions. The same tendency is recognized by Harley in the early stage of scarlet fever where there is high pyrexia.[25]

[Footnote 20: Die Bluthlumpen dann der Häutiger Bräune, Wien, 1852.]

[Footnote 21: Med. Times, vol. i. p. 23, 1860.]

[Footnote 22: Am. Journ. Med. Sci., April, 1864.]

[Footnote 23: De la Thrombose cardiaque dans la Diphthérie, Paris, 1872.]

[Footnote 24: Dict. Encycl. des Sci. méd., vol. xviii. p. 484.]

[Footnote 25: Medico-Chirurg. Trans., vol. lv.]

Notwithstanding the diminished proportion of fibrin in typhoid fever, and the impossibility of explaining, in many cases, any increase of the plasticity by local inflammatory disorders, cardiac concretions have been observed by Huss, Virchow, and Hardy.[26] Bucquoy also relates, after Huxham, an epidemic which reigned at Plymouth in 1742 amongst sailors who came from a long cruise, characterized by dyspnoea, cardiac palpitations, and intermittences of the pulse. Many of those attacked died, and at the autopsies made polypoid concretions of considerable elasticity and adherent to the walls of the heart were found. Another similar occurrence took place amongst the soldiers of the garrison of Rocroy in 1746. Quite a number succumbed after having shown symptoms similar to those of the sailors of Huxham. Cadaveric sections discovered in the left ventricle several hard, consistent cardiac thrombi.

[Footnote 26: Quoted by Bucquoy, Des Concrétions sanguines, Paris, 1863, p. 36.]

III. Endocarditis.--Whatever may be the opinion of different authors in regard to the frequency of endocarditis when intra-cardiac thrombi are present, it is certain that if it does exist the explanation of the presence of these deposits is clear and ample. In endocarditis we have both a local and mechanical cause and also a vital condition of fibrinous deposits in the heart. As a mechanical cause we know that often it is the cause of the stenoses of orifice which are present, and that further, by its effect in producing roughening or fissuring of surface, it offers a strongly predisposing cause of the {726} deposit of fibrin. Ulcerative endocarditis acts still more efficiently in this direction, owing to the fact that it produces its effects as much on the surface of the valve, aortic and mitral, near the adherent portion and in the neighborhood of the cardiac orifice, as between its layers. The result is, that the surface is rough, unequal, presenting often cauliflower excrescences, and showing sometimes, in the midst of a mass of fibrin that has become deposited by degrees, portions of a softened, partially-detached valve which was the nucleus of the outer layers of fibrin. Further, endocarditis of both forms acts as a vital and efficient cause of cardiac thrombosis, in that it belongs to the class of inflammatory diseases which occasions an absolute increase in the proportion of fibrin of the blood (from 25/1000, concrete fibrin 3, and metalbumen 22, to 56/1000, concrete fibrin 17, metalbumen 36); and also, more especially in ulcerous endocarditis, by the transport of infectious materials into the blood, which still further tend to cause coagulation.[27]

[Footnote 27: At times there is complete deprivation of epithelium over a limited area, and in rare cases slight ulcerations of membrane. These two conditions are efficient factors of the exudation of plastic lymph.]

SYMPTOMATOLOGY.--According to Laennec,[28] it is equally erroneous to attribute to cardiac thrombosis many symptoms which properly belong to an organic lesion of the heart (notably hypertrophy) as it is to believe that intra-cardiac thrombi never begin to form until the terminal period of life. According to him, Haller, Vinckler, Staneari, and Bonaroli[29] have observed obliterations of the internal jugular vein and carotid artery by very firm concrete fibrin, and he himself has seen a similar production in the inferior vena cava for the space of four fingers' breadth. Although these concretions were evidently formed during life, they occasioned no symptoms indicative of their presence, nor were there any obstructions in the course of the circulation which could explain their origin. Reasoning from these facts and from the phenomena which occur in aneurismal tumors, it seems highly probable that the blood should coagulate in the heart also during life. Later writers frankly admitted that coagulations in the veins caused partial dropsies, a usual instance of which is the white swelled leg, or phlegmasia alba dolens, from obliteration of the femoral vein.[30] This is not invariable, for I have seen, in patients who have succumbed to diphtheria, both venæ cavæ obstructed by coagula, without having observed during life either local or general oedema.[31]

[Footnote 28: A Treatise on Diseases of the Chest, p. 183, Philada., 1823.]

[Footnote 29: Quoted by Morgagni, Epist. 64.]

[Footnote 30: Vide Bouillaud, Archiv. gén. de, Méd., t. ii. et v., quoted by Hope.]

[Footnote 31: Thrombose cardiaque dans la Diphthérie, Paris, 1872, p. 43.]

Scarcely any contemporary author doubts that cardiac thrombosis gives rise to more or less well-defined symptoms. What these are we shall now consider. Of course we are far less liable to-day, when the diagnosis of organic cardiac disease is so accurate, to attribute to intra-cardiac thrombi the signs, physical or rational, which properly belong to them, and which ancient observers could not differentiate. Nevertheless, there are complex cases in which one is at fault even in regard to this problem.

The symptoms of cardiac thrombosis vary naturally with their size, situation, and rapidity of formation. Certain authors have affirmed, for example, that the concretions formed in an auricle cause a greater amount of interference with the circulation than those elsewhere situated. This they do partly by reason of their size and the less contractile power possessed by the auricle, partly because from the auricle prolongations are sent off which occlude the cardiac orifices. When cardiac concretions form suddenly a few days previous to death, they always aggravate all the symptoms of an obstructed circulation.[32] If the case be one of pre-existing disease of the heart, they soon obliterate the cardiac cavities and lead to a rapid fatal {727} termination. According to Grisolle,[33] when the concretions are small and form an obstacle neither to the play of the valves nor to the cardiac circulation, they are not revealed by any appreciable functional trouble. The opinion of Grisolle in regard to small coagula is also shared by Legroux, especially when they are fixed at a distance from a cardiac orifice or concealed in a sinus. When, however, the thrombi are larger and interfere more or less with the course of the blood, they occasion very marked symptoms.

[Footnote 32: Hope, On the Heart, p. 486, Philada., 1846.]

[Footnote 33: Pathologie interne, p. 467, Paris, 1865.]

Even before the days of auscultation there were certain rational signs which were dwelt upon with much force as showing the presence of cardiac concretions. Thus, Senac[34] writes that the patients thus afflicted feel a weight or oppression in the præcordial region which sometimes becomes extremely painful. Palpitations and irregularities of the pulse were also noted as symptomatic of these productions. Laennec believes that coagula of any size may be recognized; "when, in a patient who till then had presented regular pulsations of the heart, these suddenly became so anomalous, confused, and obscure that they can no longer be analyzed, we may suspect the formation of a polypous concretion."[35] He further adds that if the trouble takes place on one side alone of the heart, the fact is almost certain. When the coagula occupy the cavities of the right heart, the sounds of the left heart may remain normal whilst those of the right side are more or less distant and muffled (Legroux). Several authors, amongst whom we should mention Legroux, Bouillaud, Barth, and Roger, have mentioned amongst the physical characters which show the existence of intra-cardiac thrombi the sudden development of a blowing murmur limited to the præcordial region or propagated into the aorta. Sometimes this bruit was soft, sometimes harsh and rough. These writers have also noticed, in conjunction with grave general symptoms, the doubling of the first sound of the heart, making occasionally a sort of galloping murmur. As regards the recognition of concretions on one side alone, I acknowledge that after auscultating carefully several cases in which the autopsy showed coagula formed during life, I have been unable to note signs sufficient to justify a differential diagnosis.

[Footnote 34: Traité de la Structure du Coeur, de son Action et de ses Maladies, t. ii. p. 470 et suix, quoted by Bucquoy.]

[Footnote 35: De l'Auscult., t. ii. p. 597, quoted by Hope.]

The distinction appears to me difficult in like cases, for how explain that a trouble so considerable, even though it exists on one side only, should not influence the entire cardiac circulation? Moreover, it should be emphasized that the phenomena dwelt upon do not always manifest themselves when the cardiac contractions are perfectly normal. The heart-beats may be increased in frequency and the rhythm be changed. The passage, therefore, from a state of relative calm merely to that of extreme agitation is appreciated less readily. This is particularly true of the symptoms usually described as pertaining to the presence of terminal coagula. For here, at a period approximating the fatal termination, it is wellnigh impossible to determine accurately special symptoms. For this reason it is not surprising how authors have varied in their descriptions, and at best none of them are completely full and satisfactory. I have myself many times sought to recognize the blowing murmur given by Bouillaud as a physical sign of cardiac concretions, but in not a single instance have I been able to satisfy myself as to its existence. True it is that the cases I have watched with greatest care were those of children affected with toxic diphtheria, and it is possible, on account of the infrequency of valvular diseases during childhood, that more than once there may have been confusion between the signs afforded by newly-formed thrombi and those which belonged exclusively to a pre-existing disease of the endocardium.

Moreover, these murmurs have been heard and described by too many good {728} observers (Walshe, Flint, Richardson) for any small negative evidence to weigh against that which is very positive. Sometimes they have been but the exaggeration of a bruit previously heard and which characterized an organic affection of the heart. Sometimes the presence of the thrombus has caused the diminution or complete disappearance of the pre-existing structural murmur. Again, these murmurs are discovered for the first time when the other signs indicate the existence of intra-cardiac thrombi. When they are heard under these circumstances they prove positively that the coagula have sent prolongations between the cavities of the heart or into the great vessels, so as to prevent the accurate coaptation of the valves or to obstruct the onward current of the blood. In the first case a regurgitant murmur is occasioned, tricuspid or mitral, which is heard at the apex; in the second case a basic murmur is detected, which is pulmonary or aortic. Usually these murmurs are systolic, although they may in rare conditions be diastolic. The murmurs have been heard more frequently on the right side of the heart, and have pointed by their location of greatest intensity to the obstruction of the infundibulum and pulmonary orifice. They are then basic or suprabasic, and carried upward toward the infra-clavicular region on the left side. These murmurs are heard very rarely on the left side--so infrequently, indeed, that Walshe cannot affirm that he has ever observed clinically one in this region. Theoretically, of course, such murmurs may be heard at any spot in the præcordial region, and with the first or second sounds provided their size and position in relation to orifices or valves could sufficiently account for them. Whilst there can be little question that murmurs do take place in the præcordial region wholly due to the presence of heart-clot, it is probable that their frequency and diagnostic importance is less than superficial consideration would cause one to believe. Thus, Flint[36] states that "the presence of coagula may occasion an endocardial murmur, but as a rule it is wanting, probably in consequence of the enfeebled action of the heart." Richardson[37] holds an analogous opinion, and writes: "There are sometimes abnormal sounds, but it is difficult to distinguish these from murmurs the results of valvular lesions." Walshe[38] is at variance with this view, especially in regard to the thrombal de novo murmur, and has "heard such a murmur when the examination post-mortem showed the fibrinous coagulum as the only probable cause of it."

[Footnote 36: Disease of the Heart, Philada., 1870, p. 280.]

[Footnote 37: The Coagulation of the Blood, Lond., 1858, p. 428.]

[Footnote 38: Op. cit. (foot-note).]

Auscultation.--In the heart the single, constant sign that has been observed consists in the modified tonality of the normal sounds. These are rapid, irregular, muffled, obscured, and distant. There is notable inequality also in the strength of successive beats, which is obviously explained by the great difficulty the blood encounters in traversing the heart. Now, as I have seen in frequent autopsies that the valvular mechanism of the left heart is ordinarily free from any fibrinous deposit, it is readily understood that it can produce the two sounds normal as regards situation and time, but greatly modified in transmission.

Percussion.--Percussion, except in particular cases which are rare, and in which the cavities are much distended by their contents, will only furnish us with negative signs. When the ventricles are swollen by large coagula, the percussion dulness will be extended laterally. As the right cavity is usually the seat of the deposit, it will be particularly marked toward the right of the sternum. In those instances where the area of præcordial dulness had been determined before the formation of the fibrinous concretion this extension becomes a physical sign of great value. It is to Piorry and the use of the plessimeter that we owe whatever of exactness belongs in like cases to this method of examination.

{729} Inspection and Palpation.--The cardiac impulse may be unaffected by the presence of the thrombus, and if it has been regular in rhythm previous to its formation it may still remain so. This condition is infrequent, however, and usually the pulsations become irregular, tumultuous, and rapid. The force of successive beats will also be different. These signs can be determined by the sight and touch.

Pulse.--The characters of the pulse are variable. Sometimes it presents manifest inequalities, occasional intermittences, and is extremely frequent. It may be quite feeble in the beginning, and afterward gain in strength. Sometimes, in spite of its weakness and depressibility, it retains its regularity and its rhythm is unchanged. The coagulum existing in the cardiac cavities, especially on the right side, explains the variations of the pulse. Effectively, at each contraction of the ventricle this chamber, containing a less quantity of liquid blood, projects a smaller amount of venous blood to the lungs. Besides, this quantity is insufficient to replace the volume of revivified blood which leaves the lung with each inspiration. Soon the left cardiac cavities contract with but small power upon an amount of blood below the normal, and yet it is with this supply that the left heart must satisfy the needs of all the organs. The arteries during life become nearly empty, and it is to this condition, as well as to the lack of synchronism between the action of the two sides of the heart, should be attributed the signs we recognize in the characters of the pulse.[39]

[Footnote 39: Robinson, loc. cit.]

In some instances of cardiac concretions the sonority of the chest remains normal. In others it is obviously augmented, and even by percussion very lightly performed a sound of raised pitch is produced. According to Richardson, this acute emphysema is the direct result of an insufficient blood-supply in the capillaries which surround the pulmonary alveoli. Whilst such a condition may often be observed amongst children, it is not unknown with adults. The affirmation, therefore, of Walshe, that it can only be observed in young people, and that in adults its place is supplied by considerable pulmonary congestion, is not exact. Since Richardson first called attention to the exaggerated sonority of the lungs in cases of heart-clot, other observers have also referred to it. Lavirotte (1864) particularly has insisted on it as a proof of fibrinous deposition in the right heart, and has demonstrated with pathological specimens that it was occasioned by the exsanguinated state of the lungs and their hyperdistension with air.[40] On the other hand, Raynaud[41] states that when the left cavities are the seat of the concretion there is considerable stagnation in the lungs, and they show signs of great congestion. Thoracic percussion becomes less resonant, and subcrepitant râles are heard in an extended area. Sometimes, even, a moderate hæmoptysis takes place. These signs of emphysema on one hand, of congestion on the other, are not spoken of by the majority of writers on this subject; yet when they are present they will serve to fix our diagnosis and render it more certain. With respect to emphysema, especially amongst children, we should mention its great frequency, and on this account perhaps proper value has not been given to it when found at the autopsy of a child whose death has been occasioned by cardiac thrombosis. When the cardiac thrombus is present in the right side of the heart, Legroux[42] has shown that there will be a more or less turgid condition of the veins of the neck, and perhaps also of the right upper limb. With this distension of the veins we shall remark, according to him, a partial or general infiltration of the subcutaneous tissues. Sometimes the oedematous condition is limited to the face and neck; occasionally it extends below the diaphragm, especially on the right side of the body. The extent of the oedema will depend upon the number and size of the prolongations which are {730} given out by the main coagulum. Occasionally these prolongations have been found not only blocking up the pulmonary artery, but also filling one or both venæ cavæ and branching out as far as the jugular and subclavian veins.

[Footnote 40: Congrès Medico-Chirurgical, Lyon, 1864.]

[Footnote 41: Dict. de Méd. et de Chirurgie, vol. viii. p. 573.]

[Footnote 42: Gazette hébdomadaire, 1856.]

In my own observations I have always found the veins of the neck manifest, without in a single instance reaching any great size, and never have I noticed the prominence of the eyes noted by Walshe. In these cases cyanosis was limited, and was notable in a marked degree only upon the lips, the cheeks, and in the upper extremities. The general or local infiltration of tissue I have never remarked, although closely looked for on several occasions. Some authors, indeed, have described a bluish appearance of the entire surface of the body, together with signs of general infiltration. The explanation given of these phenomena is that there is a general obstruction of the capillary circulation, and that the return of the venous blood to the cavities of the heart is rendered almost impossible. In other words, we have here a well-marked asphyxic condition. If this be true, it is only partially so, and there must be great variation in different instances of fibrinous deposition in the right heart. The rational symptom which was for me one of great value in the diagnosis of these cases was that of excessive pallor, not only of the face, but of the limbs and the entire trunk. This pallor appeared constantly to increase until the last moments of life.

Richardson indeed says the symptoms are those of syncope, not of asphyxia. The different processes of life are arrested on account of a simple absence of arterial blood, not owing to the presence of blood unfitted to reconstitute the tissues. The tendency to fainting is probably due, therefore, to the fact that the right ventricle being more or less completely filled by a fibrinous coagulum, the blood is prevented from passing through its cavity and entering the lungs. As a result, there will be but a relatively small portion of blood which becomes oxygenized after each right cardiac systole. When the clot occupies the left ventricle and auricle, there will be a reflux of blood into the pulmonary tissue, thus causing great congestion of this structure. So intense will this congestion become that occasionally hæmoptysis results and pulmonary apoplexy may be developed, due, doubtless, to rupture of the capillary vessels. This condition occurs before the right heart is much or at all obstructed by coagula. We can appreciate that the physical signs must, if properly noted, show manifestly in which cavity the clot is located. If it be in the right heart, anæmia and emphysema of the lung should follow; hence breathlessness and increased pulmonary resonance. If it be in the left cardiac cavity, the lungs become engorged very rapidly, and we should find dulness on percussion, moist râles, and perhaps an equal or even greater amount of dyspnoea.

Difficulty of breathing appears to belong as well to the symptoms which indicate cardiac thrombus on one side of the heart as to those which characterize its presence upon the other. This symptom was first accurately described by Hope, since his day by Richardson and Meigs.[43] It has something special in its features which strikes one particularly, but may deceive unless closely observed. It takes place not because the movements of the thorax are interfered with, not because the entrance of air into the lungs is prevented, for the vesicular respiratory murmur is easily distinguished, but because the amount of blood furnished by the pulmonary artery is diminished. The anguish of the patient is sometimes terrible. The nares dilate, the chest expands spasmodically with each inspiration, and the patient is agitated, moans, and shows that extreme craving for air described by Van Swieten in the summæ anxietates. Under these circumstances, Hayden[44] states, the surface is cold, and often humid with perspiration. Pain and great oppression {731} in the præcordial region have occasionally been referred to, as in the patient of Beau, who said, in placing his hand to his chest, "I have there a weight which has suddenly formed and which stifles me." Often the anxiety is extreme, and the painful sensations continue to increase steadily until death occurs. In rare instances the suffering, when it has reached a certain degree of intensity, may remain stationary, or even become considerably less. If such a respite occur, it is only temporary, however, and the anguish soon recommences. In milder cases sometimes, and apparently after dissolution or disintegration of the clot, the severe symptoms may by degrees disappear, and from this period the patient makes a steady, uninterrupted march toward recovery. I have only lately witnessed a similar example in a youth attacked with typhoid fever, which had reached the third week. There are constant and intense effort to breathe, extreme restlessness, and the patient will throw himself from one side of the bed to the other, and scarcely remain quiet for a few moments. These symptoms are usually more developed when there is concomitant cardiac disease of organic nature, and unless this be present may not be so pronounced as to concentrate attention upon them.

[Footnote 43: Am. Journ. Med. Sciences, April, 1864.]

[Footnote 44: Cases 106, 107, and 111.]

The brain scarcely shows the effect of congestion when the patient dozes for a few moments even in the midst of his great distress. These times of repose are frequent, but very temporary. In a little while the patient goes off in delirium or has a convulsion. Again, he relapses into coma, in which state death may take place. In some instances there has been obstinate vomiting during several days preceding a fatal termination. It is possible that this symptom favored the rapid development of the thrombus.

The preceding signs and symptoms will sometimes declare themselves suddenly in the midst of an inflammatory or cachectic affection, and will then point directly to the presence of a cardiac thrombus of considerable size which has rapidly formed, and which obstructs an orifice or interferes with the normal play of the valves. Again, there are all the physical evidences of an old organic affection of the heart, or those of acute endocarditis or pericarditis, and rapidly all the symptoms referable to the heart become greatly increased, whilst orthopnoea, pallor, and coldness of the extremities take place. If a careful examination of the chest reveals no intercurrent and pulmonary nor superadded cardiac affection, we may then fairly assume the existence of an intra-cardiac concretion. Nevertheless, we should remember that in many of these cases there is a close resemblance of the symptoms with those occasioned by a sudden rupture of one of the chordæ tendineæ in the course of acute endocarditis (Walshe).

We should not lose sight of the fact that at times a clot will form in the heart without giving rise to manifest symptoms unless the attention be specially directed to its formation. This will be true in instances where the coagulum forms slowly, is small, or occupies a place removed from orifices or valves. In a cardiac sinus, for example, a coagulum of inconsiderable size may remain fixed and latent for a long period. Such is not the case, as we already have shown, when the coagulum fills in part one or more of the cardiac cavities, is situated near an orifice, is attached to the walls by a pedicle which allows it to float freely in the ventricle, or is intertwined with the valves or chordæ tendineæ. Under all these circumstances, they give rise to the signs and symptoms we have dwelt upon above, and which ordinarily make known their presence. Occasionally, however, there is such a combination of symptomatic morbid phenomena relating to different organs that we are at a loss to separate them accurately and to determine how this or that symptom is occasioned. This statement is particularly true in regard to the distinguishing symptoms which indicate the presence of terminal coagula. At a period when the fatal termination is not far removed, and when it is extremely difficult both to recognize and interpret special {732} symptoms, it is readily understood why those pertaining directly to cardiac thrombosis have not hitherto been fully and accurately described.

COURSE, DURATION, AND TERMINATIONS.--Cardiac concretions may form more or less rapidly, and in certain situations occasion death instantaneously and surely. This is eminently true of large coagula which fill up the infundibulum and pulmonary artery. Cases of this sort have been mentioned by various authors. Amongst others, we would specially direct attention to those instances in which sudden death has taken place during the puerperal state after severe post-partum hemorrhage. The patient has at times, in assuming an erect sitting posture, been attacked with a syncopal attack resulting in a few instances fatally.[45] In the same category we should include those examples in which sudden death has followed severe surgical operations.[46] Two cases of this termination, due to coagula in the right heart, are reported by Robert Lawson.[47]

[Footnote 45: Philada. Medical Examiner, March, 1849, paper by Charles D. Meigs; vide also Spiegelberg, Lerbuch der Geburtshülfe, and Lusk, The Science and Art of Midwifery, p. 597.]

[Footnote 46: Med. Times and Gazette, vol. i., 1873, p. 58; also Pathol. Soc. Trans., vol. xxvii. p. 70.]

[Footnote 47: Med. Times and Gazette, Feb. 8, 1873.]

In cardiac dilatation this mode of death is not infrequently seen. It here seems to depend mainly upon stasis of blood caused by weakened power of contractility in the right heart and "by impairment of respiratory and nutritive attraction arising from feeble respiration and arrested tissue-change" (Hayden). The post-mortem revelation has afterward shown cardiac thrombosis to be the efficient cause of death. In diphtheria[48] and pneumonia such examples are not infrequently encountered. As Austin Flint[49] remarks, however, these coagula present almost identical physical characters with those formed after death, and consequently to fix precisely the moment of their production will at best be but a matter for conjecture. According to Walshe, it would be difficult to determine whether or not some of these almost instantaneous deaths occurred as a coincidence or as an effect. Besides, it is frequently impossible to determine the length of time they have existed before completely obstructing the circulation through the pulmonary artery into the lungs, and hence causing fatal syncopal or asphyxic phenomena. Bristowe[50] goes so far even as to affirm in the great majority of cases that cardiac concretions are unaccompanied with appreciable symptoms. In this statement he includes coagula of large size entirely filling one or more of the cardiac cavities, and doubtless formed within a few hours of the final termination. To quote his own words, "We ought to require very strong testimony indeed to convince us in any case that concretions found in the heart at the time of death have caused death, still more to convince us that those clots which resemble in every point the clots which are the mere result of dying have had this effect." How different does this sound from the opinions of B. W. Richardson,[51] who attributes so many well-marked symptoms to the formation of voluminous moulded clots in the heart! And, indeed, is it not at variance with the views of a host of the best medical observers? We believe Bristowe goes too far, and that cardiac concretions are not infrequently the cause of very sudden death both in acute and chronic diseases.

[Footnote 48: Robinson, loc. cit.]

[Footnote 49: Diseases of the Heart, p. 276, Philada., 1870.]

[Footnote 50: Reynolds's System of Medicine, vol. v. p. 113.]

[Footnote 51: "Lectures" in the British Medical Journal, 1860.]

There are numerous instances in which the coagulum formed in the heart is of smaller size, does not form so rapidly, and besides occupies a position in which, as it does not interfere greatly with the function of the heart, death does not of necessity immediately take place. Little by little, however, the clot is added to, and before many days have elapsed symptoms of gravest {733} import are pronounced. So usual is it for the phenomena connected with the formation of a large cardiac concretion to be accompanied by those which properly belong to another serious affection which may likewise occasion rapid death (pneumonia, endocarditis, typhoid fever, diphtheria, etc.) that we with the greatest difficulty separate the symptoms, and can assign to the intra-cardiac condition those doubtless occasioned by it.

The cases referred to above are not the only ones. Occasionally we meet with cardiac concretions after death which have evidently existed for a number of years, and sometimes without having ever revealed their presence by notable interference with the circulation or in any way affecting the habitual good health of the individual (Laennec[52]). This is perhaps not to be wondered at when the coagulum is small and situated near the apex of the heart, in one of the auricular appendages, or in such a position as not to alter the play of the cardiac valves or obstruct the orifices. But when we see a whole cavity, as an auricle, forcibly distended by an old concretion which fills its entire cavity, the absence of all symptoms during life pointing to its existence occasions much surprise. Some of these large coagula have nevertheless, by a sudden change in their position, caused instantaneous death; others again, after giving rise to obscure symptoms affecting both the pulmonary and cardiac circulation, have likewise brought about a rapidly fatal termination.[53] Sometimes, in consequence of the condensation or atrophy of the clot, the phenomena which took place suddenly with great intensity and indicated its presence became gradually modified, and we have known one remarkable instance in a youth during the third week of an attack of typhoid fever where the accidents thus occasioned completely disappeared, and the patient left the hospital apparently cured.[54]

[Footnote 52: Dict. Encycl. des Sci. méd., article "Concrétions sanguines."]

[Footnote 53: Edin. Med. Journal, April, 1868, v.--case by H. Douglas.]

[Footnote 54: What occurred in this case I am of course unable to state in a positive manner. All I know is, that the heart became suddenly obstructed, followed by weak, irregular pulse and dusky countenance, and that in twenty-four hours, under treatment with frequently-repeated doses of digitalis and carbonate of ammonia, the accidents subsided. Was there a solution and disintegration of an incompletely formed heart-clot? It seems to me probable.]

COMPLICATIONS AND SEQUELÆ.--One, if not the gravest, complication which can arise during the formation and duration of heart-clot is the production of an embolus of the pulmonary artery, completely filling up its cavity, arresting respiration, and causing sudden death by asphyxia. More frequently smaller portions of heart-clot become detached and are transported farther along by the blood-current. Finally, they become arrested in vessels of smaller calibre. In these they may remain for a short time, and then become dissolved and resorbed, leaving the calibre of the vessel free after their disappearance, or else they form permanent plugs and give rise to inflammation, coagulation, or hemorrhage. According to the investigations of Lefeuvre,[55] which are both clinical and experimental, it would appear that the obstruction of the arterial distribution to any given part is almost immediately followed by engorgement of tissue and hyperæmia of the capillaries of the affected region. Feltz[56] has further shown that this condition is brought about by reflux from the veins and paralysis of the capillaries. It is not infrequent, moreover, to find hemorrhage into the tissues as a direct sequela of this changed condition of circulation.

[Footnote 55: Brit. and Foreign Med.-Chir. Review, Oct., 1871.]

[Footnote 56: Traité clinique et expérimentale des Embolies capillaires, Strasburg, 1870.]

These are, in fact, the conditions described under the name of infarctions. Small detached particles may be detached from the cardiac clot, if it be found in the left cavity, and transported after a similar manner by the blood-current of the aorta and its divisions until finally arrested in the different {734} viscera of the economy (spleen, kidney, liver) or in the arteries of the extremities.[57] In these different situations they give rise, when finally arrested, to results which differ considerably according to the structure of the organs or tissues where they become impacted. In certain instances, carefully studied by Senhouse Kirkes, the disintegrated and puriform contents of old fibrinous coagula are carried throughout the vascular system and determine marked typhoid phenomena. The patient is attacked with irregular paroxysms of fever of intermittent type, diarrhoea, vomiting, and extreme feebleness. Kirkes explains these symptoms partly by the obstructions occasioned by small emboli, partly by a sort of poisoning due to the transformation of the fibrin. The accidents thus occasioned at times very closely resemble those which characterize pyæmia.[58] The fluid contained in the interior of the old clots, which give rise to these accidents by reason of their transformation, is thick, grumous, and puriform. It is surrounded by a sort of pseudo-cyst, and is composed mainly of altered fibrin and red and white blood-corpuscles.[59]

[Footnote 57: Gazette hébdomadaire, 1856. Legroux reports a case of acute rheumatism accompanied by endocarditis and followed by concretions in the left cavities of the heart, and obliterations of the arteries of the limbs by emboli without gangrene ensuing.]

[Footnote 58: Dict. Encycl. des Sci. méd., loc. cit.]

[Footnote 59: Pathol. Soc. Transact., vol. xiv. p. 65, cases by J. W. Ogle.]

Pulmonary apoplexy and hæmoptysis often take place in connection with the presence of a fibrinous clot of the right heart. This connection, however, is not absolute, and many cases of right cardiac coagulum have been observed in which neither of these complications became manifest. When there has been pre-existent valvular disease, especially of the mitral, these sequelæ more surely follow than when there has not been this organic disease. The connection between the pulmonary apoplexy and the valvular affection is even more intimate than that of the hæmoptysis, and the same statement is also true of its relationship with cardiac thrombosis.

Upon this subject Hayden[60] writes as follows: "Pulmonary apoplexy seeming to require it as a necessary antecedent condition, while hæmoptysis, though generally associated with thrombosis in the last moments of life, frequently does occur independently of it."

[Footnote 60: Dis. of the Heart, vol. i. p. 529.]

The doctrine of Ludwig, as supported by Niemeyer,[61] that the pulmonary apoplexy is directly due to stasis and deposit in the capillaries of blood-corpuscles, does not appear possible if we accord faith to the researches of Waters,[62] who has shown an intercommunication between the bronchial vessels and pulmonary veins; and reasoning upon this basis we have a strong confirmation of Virchow's theory of hemorrhagic infarction (Hayden) consequent upon embolism.

[Footnote 61: A Textbook of Practical Medicine, 1869, vol. i. p. 156.]

[Footnote 62: The Human Lung, 1860, p. 201.]

PATHOLOGY AND MORBID ANATOMY.--In the great majority of cases clots presenting different physical characters are found in one or more of the cavities of the heart after death. According to the supposed time of their formation, they have been very properly divided into--1, cadaveric (post-mortem); 2, terminal (in actu mortis); 3, ancient (ante-mortem). It is important at the very beginning of the considerations which I shall make in regard to these formations to determine, if possible, the physical characters of cadaveric and terminal clots, so as to be able afterward to more clearly separate from them the true cardiac concretions or those formed at a time more or less removed from the period of death. Without much question, it is owing to the indifference or neglect of later writers in making these necessary distinctions that uncertainty has arisen in the minds of many with respect of the age of many heart-clots. The cadaveric and terminal clots would indeed have but slight pathological interest attached to them were it not that occasionally during {735} life, in a spontaneous manner, cardiac thrombosis suddenly takes place, and is always the cause of symptoms of considerable gravity and which often occasion a fatal termination.

I. Cadaveric Clots.--These present the characters of blood drawn from the arm by venesection and which is allowed to coagulate in a vase. 1. Sometimes they are large, soft, homogeneous, friable masses, distending one or more of the cardiac cavities, and having an appearance very similar to badly-cooked currant-jelly, and there is no apparent separation of the fibrin and the globules. Such an aspect is found particularly when the relative quantity of fibrin is below the normal or the blood is deficient in plasticity. In alkaline poisoning and many adynamic forms of disease this is notably the case.[63] It may likewise occur in forms of death in which there has been considerable obstruction to the circulation. 2. In a somewhat similar manner, when the blood is removed from the influences which give it life and stagnates, or is arrested within the heart, coagulation takes place and the blood separates into two layers. The upper is fibrinous, and resembles the buffy coat covering a clot after bloodletting; the under layer is mainly cruoric, and encloses within its meshes by far the larger proportion of the red globules. This latter mass always forms the lowest stratum by relation with the position of the body after death. Between these two layers, and from the fact of their smaller density, we find more of the leucocytes. This formation of blood-clot in distinct strata has been accomplished experimentally by Pasta,[64] who poured some blood of an animal into the heart of an ox and allowed it to deposit. The cruoric mass is always soft, and may be readily washed from the fibrin by a stream of water. Frequently these clots distend the cardiac cavities to such an extent that when they are opened at the autopsy a portion will fall upon the table and the rest is readily detached from them. The microscope shows the same condition of globules and fibrin in these coagula as it does in those of a venesection. According to Walshe, these cadaveric coagula are usually voluminous, jelly-like masses of fibrin of a pale straw-color, semi-transparent, and containing a quantity of serum in their meshes. Never do they show the slightest signs of stratification, and are not really adherent to the cardiac walls. Occasionally their prolongations may be intertwined amongst the papillary muscles and fleshy columns. According to Legroux, it appears difficult to understand how these large masses of fibrin become separated from the blood and deposited in the heart during life, and yet he is indisposed to regard them as a strictly post-mortem production. They are for him simply the result of the agony.[65] After death the serum is expelled from the clot in larger or smaller quantity, and for a longer or shorter time according to its own spontaneous retractility.

[Footnote 63: Magendie, "Lectures on the Blood," Lancet, 1839.]

[Footnote 64: Dict. de Médecine, t. viii. p. 560, Paris, 1868.]

[Footnote 65: Gaz. hébdomadaire, 1856.]

There are instances in which death has taken place very suddenly (chloroform, lightning, blow on epigastrium), and the blood remains liquid in the cardiac cavities and shows no tendency to coagulation (Walshe). The intimate cause of this condition is difficult to state, although the sudden shock to the nervous system is doubtless the main explanation. Under these circumstances the lining membrane of the heart is apt to become stained with the coloring matter of the blood.[66] At times the ventricles of the heart contain no blood at the autopsy. This is more frequently true of the left than of the right ventricle. Even then the auricles are more or less full.

[Footnote 66: Bristowe, in Reynolds's System of Medicine, vol. v. p. 106.]

II. Terminal Clots.--These clots are found at a period more or less removed from the time of death. It may be that they have been present in the heart many days before the fatal termination is reached, or indeed that the act of dying, when the whole organism is overcome by the {736} numerous conditions which inevitably tend in this direction, is mainly instrumental in their rapid production. Of course their outward aspect as well as their intimate structure will vary greatly with their age and with the disease which has been present. Never are they formed entirely of cruor; frequently they are composed of a large quantity of fibrin. Their coloration varies with the quantity of red globules, leucocytes, and serum shut up in the meshes of the latter. In the cruoric as well as the fibrinous clots time also works changes of coloration. In the latter by the mere expression of the fibrin the coagula become less shiny and take on a darker tint, and when deeply colored by red globules they may go through many changes of tint from a violet or red-brick color to a pink. Usually, however, these latter changes require a much longer time to be effected than is properly understood in the term terminal clot. The latter is white, with a yellow or green tint, or again of a fleshy color with spots of deeper hue upon their surface. These are nothing more than small masses of blood, although to superficial inspection they may appear vascular. In structure they may be homogeneous throughout, but this is extremely rare, for in the same clot we habitually find different parts which are evidently of different ages; and not only is this true, but what leads more to confusion in regard to the precise age of a given clot is the fact that a relatively old one is at times juxtaposed or intimately annexed to a purely cadaveric one. To make the distinction of what portion of clot has been formed some time, and what part in the agony, is occasionally almost impossible. Owing to the manner of death or to certain rapid chemical changes which may take place, the interior of terminal clots is at times softened and filled with a puriform material which is probably only softened fibrin.[67] These clots are more or less firm and elastic. They adhere quite intimately by a number of roots to the walls of the heart, and are twined around the chordæ tendineæ, the musculi pectinati, and are closely attached after this manner in the depressions between the columnæ carneæ. Sometimes they send off long projections into the large vessels which proceed from the base of the heart. These latter may be cylindrical in form and fill up the vascular calibre, or appear like so many flattened and ribbon-like strips. The elasticity of these clots is made especially evident when we attempt to tear them away from the cavities in which they are adherent. They come away in small pieces, and show a rough, irregular surface where they have been torn asunder. Upon pressure the terminal clots allow a smaller or larger amount of serum to exude from their surface, according to their age and the site of their formation. If the quantity be large, the clot is much reduced in size and changes considerably its physical characters. It must be evident, therefore, that if a clot be contained in the ventricle, and be submitted for any notable length of time to active and forcible contractions, it cannot contain any large amount of serum. In the auricles near the appendages the clot does not bear any very strong outward pressure--not much more, in fact, than it would in an aneurismal sac. Clots in this situation may have existed, therefore, for quite a time before all or even a great part of their serum has exuded (Legroux). Rarely, terminal clots are somewhat stratified. The form of these clots is variable; usually flat, they may also be globular, ovoid, or thick. As they pass through the cardiac orifices they are narrowed. At a level with the sigmoid valves the full margin of the cusps is marked upon their surface, and discoid masses, formed usually almost exclusively of fibrin, fill the cavities of the cusps and are moulded to their surface. To this condition great importance has been attached as indicating the formation of the coagula prior to death. In fact, Poullet[68] has endeavored to prove irrecusably by experiments upon animals {737} that in all cases where these masses were present the clot had been formed quite a length of time during life. Raynaud,[69] although admitting the ante-mortem foundation of these imprints, nevertheless holds that they are produced in the act of dying. The author,[70] owing to the fact that he has found more than once the amount of fibrin and globules about equally proportioned in the deposits of the sigmoid sacs, considers that they may be formed after death. In this opinion he is upheld by Walshe, who goes even farther, and states that he has seen coagula filling the right ventricle, the infundibulum, pulmonary artery, and its branches, and tightly grasped by all these parts in which this mark was apparent,[71] and yet the coagulum was certainly formed post-mortem. This opinion was further sustained by more than one case observed during life, and in which the final symptoms were not at all those usually assigned to cardiac thrombosis. According to Richardson,[72] the fact that the clot is grooved upon its surface or contains a canicula through its interior is a positive proof of the passage of the blood-current, and hence of its formation during life.

[Footnote 67: Cycl. of Anatomy and Physiology, p. 114, 1848.]

[Footnote 68: Thèse de Montpellier, 1866. In this sign Poullet also endeavored to show a distinguishing feature between clots formed within the heart and those transported from one of the large veins of the extremities and arrested in the heart. Before Poullet, these sigmoid prolongations had been mentioned by Gallard and studied by Chauveau of Lyons and Gardner of Glasgow.]

[Footnote 69: Dict. de Méd. et de Chirurgie, vol. viii. pp. 562 and 565.]

[Footnote 70: De la Thrombose cardiaque dans la Diphthérie, Paris, 1872.]

[Footnote 71: V. (after Walshe) such a specimen, No. 3636 Univ. College Museum, London.]

[Footnote 72: On Fibrinous Deposits of the Heart, 1860.]

Whilst attaching a certain amount of importance to the signs just mentioned as indicating the age of a clot, Parrot[73] is disposed to consider the color, consistence, intimate attachments, and histological structure of far greater importance in determining their formation some time prior to death. Usually speaking, the terminal coagula have gone through no retrogressive changes as regards their primary elements. The red globules are perhaps paler than normal, but the fibrillæ of fibrin are still distinct and the leucocytes show well-defined nuclei and do not contain any fat-granules. These coagula, both terminal and cadaveric, are found more frequently in the right than the left side of the heart (Bouillaud). For the terminal especially the right auricle is a frequent location (Parrot). This does not coincide with the following table, taken from Legroux, and which shows the relative frequency of the products in the different cardiac cavities: In 48 cases concretions were found "in all the cavities at the same time, 8 times; the right cavities and the left ventricle, 2; the left cavities and the right ventricle, 2; the two ventricles, 4; the two right cavities, 5; the two left cavities, 3; the right auricle, 1; the right ventricle, 7; the left auricle 8; the left ventricle, 8 = 48 times."

[Footnote 73: Dict. Encyc. des Sci. méd., Paris, 1876.]

III. Ancient Clots.--There are several varieties which differ considerably in their outward conformation and appearances, and are formed at a period more or less removed from the time of death: (a) Stratified coagula, which are attached intimately to the cardiac walls, and present frequently an aspect which has been confounded with that of true vascularization. So intimate is their adherence at times that to effect their separation the scalpel has to be used, and in the attempt the endocardium is detached. This membrane is frequently affected at the level of their attachments with an alteration of atheromatous nature. The volume of these coagula differs considerably. According to the old writers, they may have become large enough to fill the cavities entirely of one side of the heart and weigh at least a pound.[74] This is evidently an exaggeration, and coagula of this size could only be formed after death. Still, very large clots, formed some time previous to death, have been carefully described by Bouillaud.[75] These should be considered very {738} exceptional cases, and according to Raynaud[76] such masses would inevitably cause immediate death. Notwithstanding this affirmation, an ancient clot so voluminous as to fill an entire cavity has occasionally been found. Such an instance is the one referred to by Parrot,[77] where the left auricle was found distended by a stratified coagulum, whilst the other cavities were relatively empty. Generally, the volume of these clots varies from that of a walnut to that of a grain of millet. Sometimes they are flattened out, cover a large surface, and extend from one cavity into another. It is extremely infrequent to encounter a coagulum which fills more than the one-third or one-half of the cavity which contains it. These coagula have different shapes. They are ovoid, globular, sessile, pedunculated. Their number is usually in inverse proportion with their volume. When they have a certain mass and occupy the cardiac cavities they are often unique.

[Footnote 74: Cited by Bucquoy, Des Concrétions sanguines, Paris, 1863, p. 62.]

[Footnote 75: Traité des Maladies du Coeur.]

[Footnote 76: Dict. de Médecine et de Chirurgie, vol. viii. p. 565.]

[Footnote 77: Dict. Encyc. des Sci. méd., 1 Série, vol. xviii. p. 481.]

(b) Warty excrescences, which deposit generally upon the surfaces or margins of the aortic or mitral valves, although they may be found adherent to other portions of the endocardium. These warty growths or vegetations are only so in appearance, for their real structure is mainly that of fibrin. Rarely do we find them in the right heart. They have a jagged mulberry or cauliflower aspect, and adhere to an otherwise healthy endocardial lining or to points where an alteration or fissure already exists. Sometimes they are in the form of rounded pedunculated masses, as described by Laennec,[78] and have given rise to no obvious symptoms during life. These deposits of fibrin should be distinguished from morbid growths and exuded lymph. The latter may be augmented in size by layers of fibrin, and may require close inspection to clearly differentiate them. The two preceding varieties of clot are often apparently due to some constitutional dyscrasia.

[Footnote 78: "Végétations globuleuses," Traité d'Auscultation médiate, t. ii. p. 630.]

(c) Globular concretions or fibrinous cysts, the latter term being adopted on account of the well-known contents, which have a grumous or purulent appearance[79] and are of fluid consistence. They are limited by a cyst-wall, and are firmly attached to the walls of the heart either by a single pedicle or by a series of roots intertwined with the columnæ carneæ or musculi pectinati. Usually they occupy situations in the cardiac cavities somewhat removed from the direct current of the blood. The favorite situations for them are at the apex of the left ventricle or in the appendix of the right auricle. According to Rokitansky,[80] they almost invariably occupy the left ventricle, but the observations of later writers show conclusively that this is an error (Bristowe). Thus, Hayden states that in his belief the right chambers are much more frequently the seat of thrombosis than the left chambers. This difference is explained by the greater tendency to stasis in the right heart, where also there is less considerable muscular development. Of 44 fatal cases of valvular lesion, he cites 24 instances of cardiac thrombosis on the right side of the heart, and 12 instances on the left side. No case is reported by him in which the coagulum existed solely on the left side.[81] They have been found inserting upon the cardiac valves, and in this situation, owing to their pedunculated formation and varying position, have sometimes occasioned curious physical phenomena. A rare instance of this kind is cited by Walshe,[82] where, the mitral valve being perforated, the concretion caused at one time a systolic, at another a diastolic, murmur. They vary in size from a pullet's egg to that of a hazelnut, and exist singly in a cardiac cavity or are in considerable {739} numbers. When we attempt to detach them from the cardiac parietes, we frequently tear through some of their roots and leave small masses behind. When quite numerous they are also small in size, and may then be wholly lodged in the interspaces between the fleshy columns. Under these circumstances they are usually continuous with one another and extend their processes underneath the muscular bands, which are only attached by their extremities to the walls of the heart.[83] These clots have been found in the heart free of all attachments. In one such instance reported by Pitres[84] they were very numerous and were contained in all the cavities of the heart. This was a rare example. Their surface is usually smooth and the cyst-wall occasionally very thin. The cyst itself may be unilocular or divided into a number of smaller intercommunicating cavities. Occasionally, through rupture of the sac-wall, the contents have been emptied into the cardiac cavity outside. The color of these globular or ovoid concretions is buff or brick-red, and corresponds very nearly with the fluid contained in their interior. The different coloration of the contents is due mainly to the larger or smaller proportion of the coloring matter of the blood mingled with them. Sometimes these ancient concretions are covered by coagula of later formation, and it is only after close inspection that we can determine their real character. The endocardium is usually intact at their level, and rarely shows signs even of irritative inflammation. Hence we conclude that in an analogous manner with preceding forms of coagula they owe their existence to a constitutional alteration of the blood. Whilst the rule is that on section these globular concretions offer an interior consistence which is more or less softened, yet occasionally we encounter one in which the structure is homogeneous throughout, and presents very closely the appearance everywhere revealed by its external aspect. The elements, under these circumstances, of the sac-wall and the interior part of the concretion are almost identical. Under the microscope these are recognized as being mainly compound granular bodies, oil-globules, some imperfect cells, or altered blood-corpuscles surrounded by a network of fibrin. After a brief period, and in consequence of disintegration, the contents of these cysts may resemble pus and show certain differences in their constituents according to their appearance. "When white or buff-colored they consist almost solely, if not solely, of molecular matter, oil, and broken-down corpuscles, with which are frequently mixed compound granular cells and colorless acicular crystals. When presenting a brick-red or chocolate hue they exhibit, in addition to the elements just mentioned, numerous blood-corpuscles more or less altered, and consequently more or less indistinct, and occasionally also ruby-colored, rhomboidal, hæmatoid crystals."[85]

[Footnote 79: Pathol. Society's Trans., vol. xiv. p. 65 et seq.]

[Footnote 80: Path. Anat. (Syd. Soc. trans.), vol. iv. p. 217.]

[Footnote 81: Dis. of the Heart and Aorta, Part ii. p. 1020.]

[Footnote 82: Dis. of the Heart, 4th ed., p. 106 (b).]

[Footnote 83: Bristowe, in Reynolds's System of Medicine, vol. v. p. 107.]

[Footnote 84: Bull. Soc. anatomique, Feb. 5, 1875.]

[Footnote 85: Bristowe, on "Softening Clots in the Heart," Path. Society's Trans., vol. xiv.]

It is to the rupture of cysts of similar characters with those just detailed that may be properly ascribed pyæmic symptoms occasioned by the diffusion of their contents in the circulation.[86]

[Footnote 86: Ogle, loc. cit.]

Coloration.--The color of ancient coagula varies from a dull white to that of a grayish, slightly yellowish, or slate tint. These extremes of color and all intermediary shades depend upon the age of the clot, the manner of its formation, the larger or smaller number of red corpuscles shut up in its fibrinous texture, and the chemical transformations it has undergone. In order that the opinion at first formed of the age of a clot by its coloration may be of some value, it is essential that this ocular examination may be further aided by the results of microscopic investigation. Occasionally, as already stated, the ancient coagula are covered by clots of late formation, but these may ordinarily be distinguished by even slight inspection.

{740} Consistence.--Usually the ancient coagula are firm, friable, and without elasticity. They are then readily detached from their insertions by traction, and always come away in small masses. On other occasions they offer considerable cohesion, and preserve their form when we attempt to tear through or break them. The degree of friability is in proportion with the regressive alteration of their substance. Sometimes the clinical history apparently indicates that a heart-clot has remained soft during several years (Walshe). Coagula, however, which have evidently been formed for a considerable period are frequently fibrinous or cartilaginous in their structure, and a deposit of calcareous material in their interior or upon their surface is occasionally found.

Organization.--The question as to whether the coagula formed within the cavities of the heart can become organized has been variously determined. Amongst those authors who speak of the progressive evolution of the clot, some admit the possibility, others absolutely deny it. That these cardiac clots are frequently coherent, firm, fibrous, or lamellated is no proof that they may become organized, since the same features prevail in the old coagula contained in an aneurismal sac. These latter, as we are aware, are readily separated from the membranous walls which surround them, and never take on a similar structure to theirs or give evidence of a new vascular formation in their interior (Legroux). Cruveilhier, Monneret, and Robin consider these coagula to be dead structures incapable of organization. Those who believe in the possibility of the clot becoming organized support their convictions by referring to certain rude resemblances with organized tissues; yet even these (Hunter, Laennec, Bouillaud) have never established their statements by any unquestioned examples. Moreover, we should remember that formerly investigations were made in a very imperfect manner. The instruments employed were insufficient and poorly adapted to accurate research of this kind. Whenever the organization of a clot was admitted, it was in connection with a preceding inflammation of the endocardium, which itself occasioned a plastic exudation. This exudation, becoming organized, was the means, according to them, of introducing a new vascular formation into the clot. According to the later researches of Virchow, Billroth, Feltz, etc., there can be no doubt as to the vascularization at times of ancient coagula contained in the vessels. In regard to cardiac coagula, we should urge the facts of their greater size and different situation as rendering their organization very improbable. Moreover, hitherto no experimenter has injected any vascular twigs in a cardiac thrombus. To sum up: whilst it appears possible that a cardiac clot may become organized in view of what has been shown to take place in vessels, still the facts thus far closely observed do not corroborate strongly this opinion, and we cannot pronounce ourselves in an absolute manner (Raynaud). Amongst the coagula least likely to become organized are the very large ones and those connected with the heart-walls by a narrow pedicle.

DIAGNOSIS.--From the preceding signs and symptoms can an accurate diagnosis be established of the presence within the cardiac cavities of fibrinous coagula? Evidently not if these formations be of small size and be situated where they do not interfere notably with the circulation. This is eminently true of those which are formed slowly in the auricular appendix or at the apex of the ventricle. In order that even a probable diagnosis of cardiac thrombosis should be made, it is essential that the coagulum should occupy a certain space, that it should be fixed near or at one of the orifices, or interfere in a perceptible degree with the valvular play. Due consideration is always to be had for etiological conditions when these can be wholly or in part known. If, for example, there be present an acute or chronic affection of the heart, and in a sudden manner, without apparent or sufficient {741} cause, the symptoms and physical signs pointing to greater disturbance of the function of this organ become developed, we naturally suspect the formation of a cardiac coagulum. And this is true, although the signs of this production are not dissimilar to those indicating structural heart disease. Thus, the rapid development of præcordial dyspnoea, of rapid, tumultuous action of the heart, of feeble, depressible, irregular pulse, and of extreme pallor or lividity of surface, combined with coldness of the extremities and extreme anxiety, gasping for breath, and jactitation, indicate under like circumstances the formation of heart-clot. This diagnosis is further confirmed when upon listening to the respiration we find that the air enters and goes out of the lungs freely, and that there is no evidence in the lungs of any sudden obstruction or inflammatory condition. Of course it is very important for the physician to be familiar with the patient's previous condition and antecedents. If the accidents just referred to become developed without these facts being known, it would be far more difficult to make a diagnosis of cardiac clot than when the accidents take place whilst the patient is being constantly watched and when the physical state never varies without being observed and noted. If there be a venous obstruction in one of the large veins of the limbs, either at the time or prior to the formation of the cardiac thrombus, the symptoms occasioned by it will give even more significance to those which show heart trouble. The same information is also afforded by sudden obstructions in different portions of the arterial channels; and whenever these embolic transports take place they show, with tolerable certainty, the pre-existence of an intra-cardiac thrombus. As we can readily understand, it is far less practicable to make the diagnosis of a clot which develops slowly, and therefore gives rise to symptoms gradually, than of one which has manifested itself more or less suddenly.

The physical signs of cardiac thrombosis as a complication of cardiac disease are not necessarily very significative. This is true, first, because there may not be an abnormal murmur owing to the weakness of the cardiac contractions; second, because (even if it be present) the murmur may be readily confounded with one already existing which is occasioned by organic heart disease. Theoretically, the first sound of the heart should be muffled by the presence of a coagulum of any notable size which interferes with the play of the valves, but this might be also occasioned by the presence of chronic cardiac valvulitis. Still, if an abnormal murmur, harsh or soft in character, become suddenly developed over the pulmonary or aortic orifice, where it was known not to have previously existed, it is a physical sign which points with much certainty to the presence of a heart-clot. Whenever the signs and symptoms given above which show disturbance in the heart's action occur in a similar sudden manner in the course of an inflammatory or cachectic disease, such as pneumonia, cancer, or phthisis, we should properly suspect the formation of an autochthonous or embolic clot in the heart. These formations arise also, not infrequently, as an instantaneous complication in the duration of acute articular rheumatism, certain of the eruptive or acute fevers[87]--i.e. measles, scarlatina, etc.--and the puerperal state, as we have already pointed out in another portion of this article. In pneumonia, as in the other affections just mentioned, if no fresh inflammatory area either in the lungs or in another viscus can be discovered which is sufficient to explain the occurrence of new alarming symptoms of obstructed circulation, the difficulties of a correct diagnosis are much less than if organic heart disease be present. And this is particularly true because another solution of the cause of the patient's condition is less available (Flint). Besides, if it be sure that suddenly an endocardial murmur is developed where none existed previously, this sign, {742} taken with the striking rational and other symptoms referable to the heart, is one of great corroborative value as regards diagnosis. Not only does cardiac thrombosis occur under the circumstances mentioned already when we have a certain right to expect it by reason of its relative frequency, but occasionally it will become evident by its symptoms under conditions where we have no right to look for its development. In these instances it is only by a diagnosis of exclusion that we can discover the correct interpretation of the phenomena presented. In the obstruction caused by a heart-clot developed in the right cardiac cavities there is of course stasis in the systemic venous circulation in consequence of the small quantity of blood which can pass through the heart on its way to the lungs. This condition, moreover, develops a peculiar dyspnoea which has been very striking at times, and which has been particularly considered by Richardson,[88] so as to differentiate it with an analogous but dissimilar state which prevails when the obstruction exists in the lungs or other portions of the respiratory tract. In the former case if we listen carefully to the breathing the vesicular murmur is normal in quality and pitch, although of exaggerated intensity, and the dyspnoea is evidently due to the fact that the air lacks, so to speak, a sufficient quantity of blood to arterialize it. Consequently, the surface of the body is pale rather than cyanosed, and the heart-sounds and pulse are feeble, tumultuous, or notably irregular. In the latter case the lungs are congested or there is some other evident obstruction of the larynx, trachea, or bronchial tubes which prevents the entrance into the alveoli of a sufficient quantity of blood for the purposes of hæmatosis. Hence a rapidly generalized cyanosis becomes developed, the superficial veins are generally turgescent over the surface of the body, and what with the irregular, feeble action of the heart, although its normal sounds are distinctly defined, the violent convulsive movements of the voluntary muscles, the abolition of the intelligence of the patient toward the fatal termination, we have a sufficient number of signs which point distinctly to an asphyxic state. Finally, at the end of life in the former case it is the heart which first comes to a stop, whereas in the latter the lungs are the organs which are primarily arrested in their movements. These differential signs have great practical importance. Unfortunately, there are instances in which it is extremely difficult to assign in proper degree the symptoms occasioned by the heart-clot on the one hand or obstructed respiration on the other.

[Footnote 87: Keating, Am. Journ. Med. Science, Jan., 1885, p. 122, v.--an able article, entitled "Heart-Clot as a Fatal Complication in the Acute Fevers of Childhood."]

[Footnote 88: Medical Times, vol. i. p. 330, 1856.]

We have in another place pointed out this fact where at the same time there was present a membranous deposit of diphtheritic membrane blocking up the calibre of the larynx and a cardiac coagulum distending the right cardiac cavities.[89] In like manner, there may be an inflammatory complication in the lungs themselves--i.e. broncho-pneumonia--which by its sudden beginning and the rapid rise in the number of the respirations and the pulse should awaken a suspicion as to the cause of these symptoms. An error in regard to the modifying influence of this accident would be possible were it not that broncho-pneumonia, even of limited extent, reveals itself by stethoscopic signs, and, moreover, would not explain all the phenomena which arise. These are: the excessive pallor, the special kind of anxiety, the weakness and inequality of the pulse, the muffled heart-sounds, and the very rapid death. In exceptional instances, when the lungs are merely affected with hyperæmia, the characteristic signs of cardiac thrombosis are more readily recognized.

[Footnote 89: Robinson, loc. cit., p. 48.]

That form of uræmia known as the dyspnoeic or respiratory, which has been well described by Fournier, is sometimes confounded with heart-clot. Its commencement is often sudden. Soon labored respiratory action is very marked, and approximates true orthopnoea, although there is absence of {743} pulmonary lesion. From the cardiac disturbance it can be differentiated by the pulse, the cardiac rhythm, bodily pallor, and the usual evidences of kidney disease.

The distinguishing features between pulmonary embolism or thrombosis and the deposit of fibrinous coagula in the heart are extremely difficult to delineate. At times the cardiac coagula manifest their existence quite as suddenly as does pulmonary embolism. Nothing, moreover, prevents the formation[90] at a simultaneous moment of a coagula in the veins as well as in the heart. The puerperal condition, which is a predisposing cause of an excessive relative amount of fibrin, is likewise an efficient cause of both these formations. Besides, we should add, there is no reason why the fibrinous coagulum of the heart in changing position should not throw off a plug which will block up the pulmonary artery completely. To separate these conditions or to make a diagnosis between them other than one based upon probabilities is not possible.[91]

[Footnote 90: Ball, Des Embolies pulmonaires, Paris, 1862.]

[Footnote 91: Vernay, Gaz. médicale de Lyon, Nos. des 13 Mars et 22 Mars, 1868.]

We do not consider it essential in this place to go farther and make known the signs by which we shall be able to distinguish cardiac thrombosis from certain affections of the larynx, such as laryngitis stridulosa, oedema glottidis, and membranous laryngitis, or indeed from asthma or functional disturbance of the heart. It is easy, indeed, to confound this affection with organic cardiac disease, but what we have already said should enable us to make the distinction with facility. In certain infectious diseases, and more particularly diphtheria, death by cardiac paralysis has been described. In these instances there would seem to be a real impairment, functional or organic, of the structure of the pneumogastric nerves, which is accompanied by an irregular action of the pharyngeal muscles, by vomiting,[92] extreme slowness of the pulse,[93] a remittent form of syncopal attacks, and powerless action of the heart. No such combined symptoms appear in our description of cardiac thrombosis, and they are therefore sufficient, in our opinion, to substantiate the opinion of a morbid entity which can be satisfactorily explained by recognizing solely a lesion of nerve.

[Footnote 92: Jenner, Diphtheria, its Symptoms and Treatment, London, 1861, p. 42 et seq.]

[Footnote 93: Maingault, Actes de la Société méd. des Hôpitaux, 5^{ème} Fascicule, 1861, Obs. 40.]

In many examples of death by heart-clot the aspect of the patient is very much that of one who dies in the period of a collapse from cholera (Flint), the great difference between the two states consisting in the fact that in the latter there is no notable degree of dyspnoea.

The diagnosis between coagulum of the right and left side of the heart can be determined with some accuracy if strict attention be paid to the effect of the presence of the clot on the normal cardiac murmurs. If, for example, the clot is situated in the right ventricle, it is probable that by interference with the tricuspid play it will render the valvular sound occasioned by closure less distinct, and for this reason the first sound of the heart will not be heard as well to the right as to the left of the sternum. In a similar way, the diminution of sound at the pulmonary orifice in the left second intercostal space may be explained, for the extension of the concretion into the origin of the pulmonary artery will almost certainly prevent the perfect closure of its cusps (Richardson). In deposits of fibrin in the left cavities of the heart we naturally distinguish less well the cardiac sounds along the left border of the sternum than toward its right margin. We also have congestion of the lungs, owing to the fact that a smaller quantity of blood is able to pass through the partially-filled left heart. To this is added a tumultuous, irregular action of the heart and a feeble pulse. It is proper to add, however, that excepting cases of chronic organic heart disease with dilatation or {744} degeneration of the walls deposits of fibrin in the left heart are relatively very infrequent.

In cases of acute endocarditis we have no means usually to distinguish between the general symptoms of nervous shock and the physical signs occasioned by cardiac thrombosis on the one hand, and rupture of a valve or tendinous cord on the other. According to Walshe, this could scarcely be otherwise, as clotting to a greater or less extent must necessarily deposit around the spot where the tear takes place. In view of a case reported by Hammer[94] of sudden cardiac failure in which the symptoms prior to death pointed to possible intra-cardiac thrombosis, and where at the autopsy thrombotic occlusion of one of the coronary arteries was found, it is well to bear in mind the possibility of this rare occurrence. The principal features of this case were the suddenness of the collapse, pallor, slight dyspnoea, and extremely slow pulse, ranging from 23 to 8 to the minute!

[Footnote 94: Abstract of Med. Science, 1878, p. 208; Lond. Med. Rec., March 15th.]

PROGNOSIS.--The prognosis of fibrinous coagula in the cavities of the heart is always extremely serious. The gravity of the situation is, however, in some degree proportionate to their size, their situation, and the rapidity of their formation. Thus, for example, those which are spread out like a membrane over the interior surface of the heart, as has been noted after endocarditis, are of less serious a nature than those which are polypiform. As regards the polypiform concretions which we encounter singly, which are small and formed slowly, they will be so much more dangerous as the lobe held by the pedicle can become engaged in the orifices of the heart or the vessels which take origin from it.[95] Certain well-known observers, it is true, such as Bouillaud, Barth, Roger, Racle, Meigs, and Armand, have stated their belief that in rare instances these coagula may become dissolved and disappear. Indeed, we ourselves have become convinced in more than one exceptional case that the morbid phenomena manifested, both local and general, were but the evident proofs of the beginning of fibrinous deposit in the right ventricle of the heart, and yet we have seen these evidences change their characters and finally disappear under proper treatment, leaving the patients ultimately in as good health as they were previous to their formation.[96]

[Footnote 95: Armand, Des Concrétions fibrineuses polypiformes du Coeur, Paris, 1857, p. 49.]

[Footnote 96: We are more assured in regard to this possibility than we were ten years ago (v. Thesis).]

Legroux does not believe cardiac concretions can be reabsorbed, and with Cruveilhier he admits them to be dead formations. Nevertheless, he admits that fibrinous cysts may entirely disappear by a process of progressive liquefaction. Moreover, a case reported in his exhaustive article which he observed makes him acknowledge that a fibrinous coagulum may diminish, retract, atrophy, form adhesions with the cardiac walls, and thus not interfere notably with the cardiac functions.[97] The fact, however, that there may be no present suffering does not shield such a patient surely from future accidents of a serious nature brought on by his intra-cardiac condition. About the diagnosis, however, of intra-cardiac thrombi, especially when a perfect cure has been established, there always will remain an element of justifiable doubt, and particularly in those conditions where an underlying constitutional dyscrasia of grave import was present. This latter state of itself often becomes either rapidly or eventually mortal. Apart from the gravity of cardiac thrombosis in view of its evidently pernicious influence upon the heart, it is likewise a very serious affection on account of the possibility of its giving rise to embolic transports into different viscera (brain, lungs, etc.), which themselves may bring about a direct and speedy fatal termination. Even when the embolic plugs do not occasion such considerable obstructions {745} of important vascular channels as to cause rapid death, they may fill up numerous capillaries of the economy with material of a kind which shall be followed, sooner or later, by septic symptoms or those of pyæmic poisoning.

[Footnote 97: Gazette hébdomadaire, 1856.]

TREATMENT.--According to certain well-known authors, all curative treatment of heart-clot is useless (Bucquoy). Others, more sanguine, repose confidence in the internal use of alkalies, even when a fibrinous deposit in the cavities of the heart has commenced to form. A third class of observers, whilst they doubt the efficacy of any treatment under these circumstances in causing the disaggregation or absorption of an intra-cardiac coagulum, nevertheless believe we can limit the rapidity and size of its formation, and also retard the fatal termination, by giving time sufficient for adhesions to form with the cardiac walls. Richardson has proposed the administration of minim x doses of liquor ammoniæ at short intervals in an ounce of water, in order to dissolve existing coagula, and reports favorably upon its use. Gerhardt[98] counts upon better results from the use of a saline spray of bicarbonate of sodium of the strength of ½° to 1½°. This spray should be frequently inhaled, and in this manner, he believes, the heart is reached more directly and effectually. Successes are claimed by the use of this method of treatment. According to Flint,[99] the idea of giving any remedies with a view to dissolve solidified fibrin is absurd, whereas as a preventive treatment it is legitimate in circumstances where this state is likely to occur, and may even become an important therapeutic object.

[Footnote 98: Deutsches Archiv für klinische Medicin, vol. v. p. 207, summarized in the Dublin Quarterly Journal of Medical Sciences, May, 1869, p. 421, quoted by Walshe.]

[Footnote 99: Diseases of the Heart, p. 285.]

Alkaline remedies are said to have the power of holding the fibrin of the blood in solution. If this be true, they are certainly indicated to prevent coagulation. Moreover, if the fibrin in normal blood be held in solution owing to the presence of ammonia, it must be evident that this remedy is specially indicated in carrying out a secondary object of the prophylactic treatment. Bartholow[100] still maintains, however, that frequent small doses of ammonium carbonate afford the best chances of relief even when the coagulum is already formed. The latter distinguished author advises in cases which are most imminent intravenous injections of ammonia. The proportions should be one part of ammonia to three of water. The vein selected must be the jugular, and special precautions taken to avoid the entrance of air or a foreign body into the circulation. With attention to this formal indication there is little or no danger from these injections, as has been many times proven experimentally. Walshe[101] regards the use of carbonate of ammonium, combined with bicarbonate of potassium, in five-grain doses, repeated three times daily, as a mere prophylactic, but as the best, after all, we possess, and recognizes from its use the only practical outcome from the enormous sacrifices of canine life made by Magendie in his experiments to illustrate his lectures on the blood. In spite of the numerous attempts to fluidify the blood, these efforts have always remained unsuccessful (Raynaud), and Legroux, who first proposed it, in his later writings abandoned the alkaline treatment as useless. The most he affirms that can be done is to combat with energy cardiac inflammations.

[Footnote 100: Practice of Medicine, New York, 1880, p. 285.]

[Footnote 101: Diseases of the Heart, 4th ed., London, 1873.]

There is, however, a palliative medication which is indicated by the presence of the obstacles to the circulation within the heart. The general condition must be kept in view in carrying out treatment rather than the local signs. A properly combined therapeutic method in which the derivatives and counter-irritants play an important rôle offers, in Legroux's estimation, the best solacing means to oppose to the developed accidents. We must, however, maintain the patient in a quiet attitude and administer drugs which {746} shall tranquillize pain and diminish anxiety. The counsel to keep the patient absolutely at rest is of primary importance in view of the sudden fatal accidents which have frequently occurred either in getting into bed after descending from it, or in sitting up and reaching for something the patient needs. The patient should be placed in bed in a semi-recumbent position, properly supported, and arrangements must be made so that all fatigue of eating and drinking or attending to his excrementitial functions are provided against. Of course we should treat a case of cardiac thrombosis complicating a frank inflammatory condition, such as acute endocarditis, certain forms of pleurisy or pneumonia, very differently from a case in which the state is one of relative feebleness or adynamia, as in the advanced stages of diphtheria, or after profuse uterine hemorrhage during or after confinement. In the first category of cases it may be in a few rare instances that local depletion of the blood by means of leeches or venesection is still indicated, especially if the patient be one of more than usual vigorous frame. In any example of this sort it is obvious that the internal use of the alkalies, the employment of revulsives (i.e. dry cups), and counter-irritants over the chest (as previously mentioned), adjoined, perhaps, to the action on the emunctories by diluent drinks, are the means which offer us the best guarantee of success. But how shall we act with our second class of cases? Certainly, we ought not for one moment, with our actual physiological knowledge, to consider the propriety of taking blood from a patient thus affected. May we use the alkaline treatment with reasonable hopes of benefit in a curative way? Yes, if we employ certain of the stimulating salts, like carbonate of ammonium, or even this salt combined with moderate doses of bicarbonate of potassium. We should remember, however, that these drugs are intended particularly to combat the pathological condition of the blood which apparently underlies the formation of fibrinous concretions in the heart.

Against the possible fatty degeneration of the cardiac muscular fibre, or the functional or organic affection of the pneumogastrics, which predispose to or accompany the production of cardiac coagula, we must make use of digitalis in small, repeated doses, and nux vomica or some other preparation containing strychnine. I have on more than one occasion seen these agents do evident good,[102] and on this account am encouraged to urge their exhibition. With Hertz, we are not disposed to believe that digitalis, when given with a little precaution, and especially in urgent cases, is contraindicated by the danger feared by Gerhardt and Penzoldt, that it favors thrombosis of the right side of the heart and gives rise to new emboli.[103]

[Footnote 102: Loc. cit., p. 68.]

[Footnote 103: Ziemssen's Cyclopædia, vol. v. p. 326.]

It is almost needless to add that under like circumstances we should insist upon the frequent use of stimulants, like alcohol, chloroform, and ether, in the form of brandy, whiskey, spiritus chloroformi, or spiritus ætheris, or repeated doses of strong black coffee with one of the preceding preparations added to it. In regard to the prophylactic use of alkaline treatment continued during several days and in large or frequently-repeated doses, we advise against it for the reasons, first, that we do not know, in advance, the precise conditions in which fibrinous intra-cardiac coagula will form; and second, because though the alkalies have a well-known antiplastic action, they act as depressants to the general economy when employed in the manner mentioned, which is the sole method in which their internal use would be of some practical advantage.

Whenever we have in diphtheria a case in which there is at the same time obstruction of the glottis by a false membrane and clogging of the heart by a fibrinous coagulum, we should abstain from performing tracheotomy on account of its evident uselessness.[104]

[Footnote 104: Medical Times, vol. ii. p. 617.]

{747}

NEUROSES OF THE HEART.

FUNCTIONAL DISORDERS OF THE HEART'S ACTION; ANGINA PECTORIS; EXOPHTHALMIC GOITRE.

BY AUSTIN FLINT, M.D.

The neuroses of the heart are those affections relating to this organ which do not necessarily involve either inflammation or structural lesion of any of its component parts. The larger proportion of these affections may be grouped under the name functional disorders of the heart's action. The affection called angina pectoris is characterized by pain more or less intense. It is generally associated with disordered action of the heart, and also with cardiac lesions. It may, however, exist without either disordered action or lesion, and hence it is with propriety included among the neuroses of the heart. Exophthalmic goitre is invariably associated with disordered action of the heart, but it has other very marked symptomatic traits which give to it a distinctive character. The name of the affection refers to these. The cardiac disorder is, however, the most constant, and, pathologically, the most important, and therefore the affection may be considered as one of the neuroses of the heart. In this article the functional disorders of the heart's action, irrespective of angina pectoris and exophthalmic goitre, will be first considered, and afterward these two affections will receive separate consideration.

Functional Disorders of the Heart's Action.

The disorders of the heart's action which agree in respect of their functional character present marked variations as regards the manner in which the action is disordered. An account of these will be given under the name Varieties, together with the symptomatology.

VARIETIES AND SYMPTOMATOLOGY.--The term palpitation denotes a violent or tumultuous action of the heart. A type of this variety of disorder is afforded when the heart is much excited by fear or some other intense mental emotion. The fact that emotional excitement will produce in some persons notable palpitation, and in others little or no disturbance of the heart's action, illustrates differences inherent in the organ itself as regards susceptibility to disorder. These innate differences are exemplified in cases of disease. In certain persons the heart readily takes on a morbid functional disorder from causes which in other persons do not produce this effect. A peculiar susceptibility to disorder is expressed by the term irritable heart, a term introduced by DaCosta. Instead of the violence which characterizes palpitation, there may be irregularity, with notable feebleness of the heart's action. The patient often describes this variety of disorder as a fluttering of the heart. The consciousness of the disorder is less distinct than when the {748} disordered action is violent. With irregularity are generally associated increased frequency of the heart's action and præcordial distress. The degree of disorder as respects either violence or feebleness and irregularity of action differs in different cases within wide limits. Intermittence is another variety of disorder. The intermission may extend over a period of one, two, three, or more beats. It is sometimes preceded or followed by increased frequency of action, and it sometimes occurs without any other rhythmical disturbance. The patient is usually conscious of the intermittence, and it is apt to occasion great alarm, especially before the mind has become accustomed to it. The intermissions occur more or less frequently in different cases and at different periods in the same case. In the cases of palpitation in which the heart acts with violence it is not probable that the power of the heart's action is increased. The systolic ventricular movements are quick and have a spasmodic violence, without actual increase of force. The first sound of the heart over the apex under these circumstances is short and its quality valvular. The valvular element of this sound is predominant and intensified in consequence of the quickness of the systolic movements and the small quantity of blood in the ventricles when the ventricular systole takes place. Owing to the latter physical condition the range of movement of the auriculo-ventricular valves is greater and the valvular sound proportionately increased. The systolic movements of the apex against the chest-wall sometimes give rise to a ringing or metallic sound (cliquetis métallique).

A rare variety of functional disorder which has received but little attention is notable infrequency of the heart's action. The revolutions of the organ were reduced to sixteen per minute in a case reported by Thornton.[1] In 1876, I reported 5 cases, the reduction in frequency varying from 26 to 40 per minute.[2] In one of these cases there was marked intermittency, and in another case the action of the heart was irregular. With these exceptions the rhythm was regular. I have met with a few additional instances since these cases were reported. In this variety the disorder continues for several successive days, and it may be for a much longer period. A persistent infrequency sometimes remains as a sequel, recovery in other respects being complete. In one of my reported cases the revolutions were 36 for several weeks after recovery. In these instances the infrequency of the heart's action, which is sometimes a congenital peculiarity, is acquired. Hewan has reported his own case as an illustration of this fact. His normal frequency had been 72, but after a period of intense study the frequency gradually decreased, and finally remained at from 28 to 32 per minute.[3] This variety of disorder will claim distinct consideration with reference to diagnosis and etiology. It may or may not be accompanied by præcordial distress.

[Footnote 1: Trans. Clinical Society of London, vol. viii., 1875.]

[Footnote 2: American Practitioner, January, 1876.]

[Footnote 3: London Med. Times and Gazette, March, 1875.]

The more frequent varieties of disorder of the heart's action occur in most instances in paroxysms. The paroxysms differ widely in duration as well as in their intensity. They may last for an instant only or for many continuous days. Exceptionally the duration is much longer. I have known a persistent and very great increase of frequency of the heart's action with irregularity, and such a degree of weakness that the pulse could with difficulty be counted, to continue for several weeks, leading to oedema of the lower limbs, prostration, and pallor, so that the patient's appearance was that of one moribund. In this case before the attack and after recovery there was no evidence of any other affection than functional disorder of the heart, and to this the patient had long been subject. In another case an extremely irregular action of the heart continued unceasingly for more than two months, there {749} being no signs of either an inflammatory or a structural affection of the organ, and the functional disorder at length ceasing. As a rule, an attack of functional disorder of the heart implies a liability thereto; other attacks occur after variable intervals. This fact involves a peculiar susceptibility, or, in other words, an irritable heart.

The symptoms referable to the heart may be combined with those of coexisting affections. Disturbances of digestion are frequently associated. Paroxysms of disordered action of the heart are often accompanied by gastric flatulence, and gaseous eructations afford relief. Patients are apt to endeavor to eructate by voluntary efforts. Other evidences of indigestion are not infrequent. The mind is much disturbed, especially if previous paroxysms have not occurred. The facial expression shows anxiety. The apprehension is of organic disease of the heart and of sudden death. This apprehension is excited in a marked degree by intermittence of the heart's action. It is often extremely difficult to convince patients of the absence of immediate danger. They require to be assured of this fact over and over again, and whenever a paroxysm occurs. This statement applies even to medical men who suffer from functional disorders of the heart's action. The surface is usually cool or cold. It is sometimes bathed in perspiration--a symptom probably due, in a great measure, to the condition of mind. Exclusive of angina pectoris, paroxysms of functional disorder are not attended by præcordial pain. The paroxysms may cease either suddenly or after a gradual improvement. The cessation is abrupt in the instances in which the paroxysms last but an instant or but a few moments, and not infrequently when the paroxysms are of much longer duration the normal rhythmical action is at once resumed.

The variety of disorder characterized by diminished frequency of the heart's action is often associated with cerebral disturbance. In 2 cases cited in my paper there were severe epileptiform seizures, together with frequent epileptoid attacks; in 2 cases there was mental excitability amounting to delirium; and in 1 case there was great mental and physical prostration with gastric irritability, the latter due apparently to cerebral disturbance. In 1 case only there was no evidence of disorder of the brain. Of 3 cases which have fallen under observation since the publication of my paper, in 1 there was notable mental disturbance, the mind remaining intact in the other 2 cases.

DIAGNOSIS.--Certain facts pertaining to functional disorders of the heart's action in their ordinary paroxysmal forms render the diagnosis probable. One of these is the occurrence in paroxysms, the action of the heart being normal in the intervals. Another fact is the occurrence of the paroxysms at night oftener than in the daytime. The ability of the patient to take active exercise without exciting a paroxysm and without discomfort is evidence that the paroxysmal affection is functional. A diagnostic feature of a purely functional disorder is great apprehension connected with the disordered action of the heart. The patient is apt to feel that there is imminent danger of sudden death. So strong is this apprehension that it is sometimes difficult to overcome it by positive assertions of the absence of danger. On the other hand, disordered action of the heart, when incident to structural affections, occasions comparatively little mental disturbance; the patient suffers chiefly or exclusively from the physical ailments. In a purely functional affection the patient generally is vividly conscious of the disordered action, whereas the action in structural affections may be greatly disordered and the patient take no cognizance of it. The existence of certain causes to be mentioned under the head of the Etiology bears upon the diagnosis. The liability to functional disorders, as evidenced by previous attacks, is also to be taken into account. These facts, however, are not fully adequate for the exclusion of {750} structural affections of the heart. Moreover, the persistence in some cases of notable disorder for days, weeks, or even months, would seem to render highly probable the existence of some structural affection. The basis of a positive diagnosis is the exclusion, by the absence of their physical signs, of inflammatory affections and lesions of structure.

The physician who undertakes to diagnosticate functional disorders of the heart by symptoms alone--that is, without physical exploration--must often be in doubt, and if not prudently distrustful of his ability as a diagnostician, he is liable to commit errors which are sometimes extremely unfortunate. I was requested to see a young woman who was represented as suffering from a disease of the heart from which she might die at any moment. It was stated to me that her situation was perfectly understood by herself and her family, and that the object of my visit was simply to satisfy some of her friends. I found her in a dark room, with every arrangement to prevent the least mental excitement and physical exertion. Fearing that my questions and the examination of the chest might occasion disturbance which would prove fatal, it was proposed that one of her family be made the medium of the former, and that the latter be dispensed with. This was of course objected to on my part. My questions she answered in a feeble whisper. The examination of the chest showed the absence of all physical signs of disease. The affection was purely functional and wholly devoid of danger. I could cite from cases which have come under my observation not a few in which the error of imputing functional disorders to organic lesions has occasioned the loss of years as regards the duties and pleasures of life, together with the unhappiness incident to living in daily expectation of sudden death. With a degree of practical knowledge of auscultation and percussion sufficient to recognize the signs of inflammatory and structural diseases, and self-confidence sufficient to decide upon the absence of these signs, there is but little liability to error in the diagnosis of functional disorders.

If the apex-beat be in its normal situation, and the areas of the superficial and deep cardiac regions be not extended, the heart is not enlarged; and if there be no endocardial murmur it may be inferred that the valves and orifices are normal. The exclusion of structural lesions under these circumstances is almost positive. It is open only to the exception that certain occult lesions may exist, such as fatty degeneration and obstruction of the coronary arteries. Aside from the infrequency of these, the history and symptoms may render their existence extremely improbable. A hæmic murmur at the aortic or the pulmonic orifices or at both orifices is not uncommon. That the murmur is inorganic may generally be determined by other evidences of anæmia, by an arterial murmur in the neck, and by the venous hum. With the results of physical exploration as just stated, whatever may be the form of disorder, whatever may be its intensity, whatever may be its duration, and whatever may be the associated symptoms, it may be declared to be purely functional.

The diagnosis is less simple and easy when functional disorders occur in connection with structural lesions, but without any relation of cause and effect. Lesions affecting the valves or orifices of the heart often exist without giving rise to any appreciable disturbance. They are either innocuous or their effects do not occasion any inconvenience of which notice is taken. How often is it that an examination of the chest reveals the signs of cardiac lesions which had not been suspected by either the patient or the physician! How often are applicants for life insurance astonished when told that they are not insurable on account of the signs of a cardiac affection! Now these persons are liable to functional disorders of the heart from the causes which produce them in those with perfectly sound organs, the cardiac lesions having no part in the etiology, but perhaps contributing to render the disorders more {751} intense. The problem of diagnosis in these cases is to determine that the functional disorders are not dependent on the lesions. Were they thereon dependent they might denote grave disease, but if not thus dependent they have little or no gravity. This diagnostic problem is to be solved, in the first place, by attention to the inquiry whether the lesions are in proportion to the disturbance of the heart's action. Valvular lesions, if the heart be but little or not at all enlarged, are either innocuous or occasion small inconvenience. This fact will often suffice for the solution of the problem. Moreover, the physical signs may show that the lesions involve neither valvular insufficiency nor obstruction, or, at all events, not in a degree adequate to account for the disturbed action; in the second place, the symptoms are to be considered with reference to the inquiry whether they belong to the clinical history of structural affections or of functional disorders; and, in the third place, the existence of any of the well-known causes of functional disorders is to be taken into consideration. The error is not uncommon of attributing functional disorders to coexisting lesions when the connection is one of mere coincidence. This error may be as unfortunate as that of supposing that functional disorders denote structural affections when the latter are entirely wanting.

Certain considerations, aside from the exclusion of organic affections of the heart, apply particularly to the diagnosis of that variety of functional disorder characterized by infrequency of the heart's action. It is to be ascertained that the infrequency is not a normal peculiarity, either congenital or acquired. Napoleon the Great was a well-known instance of normal infrequency, the number of beats being 40 per minute. As a rule, if an intelligent adult person has habitually a notably infrequent pulse he becomes acquainted with the fact, and therefore if he be ignorant of such a peculiarity it may be inferred that it is not normal.

There is a curious form of functional disorder which would lead to the error of inferring infrequency of the heart's action from the pulse. The disorder is characterized by the regular alternation of a ventricular systole giving rise to a radial pulse, with one too feeble to be appreciated at the wrist. Assuming the number of ventricular systoles to be 70 per minute, in such a case the radial pulse would be 35 per minute. I have met with several examples of this form of disorder in which, as may be said, there is a regular irregularity of the heart's action. The carotid pulse in these cases represents each ventricular systole, and on auscultation of the heart's sounds there will be found to be four sounds to each radial pulse. This form of disorder is liable to lead to the error of supposing reduplication of both the first and the second sound of the heart. It is hardly necessary to add that in cases of obstructive and regurgitant lesions with feebleness of the heart's action the diminished quantity of blood expelled from the left ventricle, with some of its contractions, may be too small to produce an appreciable radial pulse. The existence of these cardiac lesions is easily ascertained by auscultation.

Infrequency of the heart's action is a well-known symptom in cases of injury of the skull and in certain intra-cranial affections. Cerebral hemorrhage, embolism, and thrombosis are easily excluded by the absence of paralysis, but the exclusion of subacute or chronic meningitis is not so easy. But infrequency of the heart's action, when a symptom of the latter affection, is accompanied by cerebral symptoms denoting compression of the brain--symptoms which are wanting when the infrequency is the characteristic of a functional disorder of the heart's action. Moreover, the absence of fever, of increased sensibility to light and sounds, and of the symptoms embraced in the clinical history of cerebral meningitis, will render the exclusion of that affection positive. The heart's action is abnormally infrequent in some cases {752} of cholæmia and of uræmia, but these affections are easily excluded. Certain drugs--namely, aconite, digitalis, and veratrum viride--diminish the frequency of the heart's action. These drugs, given to a person in health, produce, in fact, a transient effect which is equivalent to the functional disorder of the heart thereby characterized.

PATHOLOGY AND ETIOLOGY.--The neuroses of the heart are functional disorders involving the relations of this organ to the nervous system. The functional disorders of the heart's action affect the frequency, the rhythm, and the force of the cardiac movements. The pathology of these disorders would be more fully understood were our knowledge of the physiology of the heart's movements more complete. We know that contractions of the heart continue when it is separated from all its nervous connections and after removal from the body, especially in cold-blooded animals. The rhythm, frequency, and force of its normal movements are evidently dependent on influences derived through the sympathetic and pneumogastric nerves. Experiments show that the movements continue, but with increased frequency and with irregularity, after division of the pneumogastrics; hence this nerve is regarded as exercising an inhibitory and regulating influence over the action of the heart. Disorders of the heart's action from causes which pertain to the brain doubtless involve especially this nerve. Other causes act through the relations with the different organs of the body by means of the sympathetic system of nerves.

Impoverishment of the blood occasions disorder, probably by affecting the nutrition of the heart. Toxical agents in the blood enter into the pathology in certain cases.

The etiology of functional disorders of the heart's action involves, as an important factor, a predisposition inherent either in the organ or in its nervous connections. A peculiar susceptibility to the causes which induce disorder is an idiosyncrasy. Causes which produce disorder in those who have this idiosyncrasy are inoperative upon others. Some persons are liable to functional disorders of this organ all their lives, whereas some appear to be exempt from any liability thereto. In this respect the cardiac muscular fibres are analogous to those of the pulmonary bronchi. A peculiar susceptibility of the latter is requisite for the capability of having bronchial asthma. The susceptibility of the heart-muscle varies in different persons, and a reasonable supposition is that in proportion to the degree of this susceptibility will the causes of functional disorder be more readily and actively operative.

Clinical observation furnishes evidence of various causes giving rise to functional disorders of the heart. The more prominent are--over-exertion of the faculties of the mind, prolonged mental anxiety, the use of tobacco, tea and coffee taken in excess, too great indulgence in venery, the unnatural abuse of the sexual system, dyspeptic ailments, uricæmia, and anæmia. These causes are often combined in individual cases. With reference to effective treatment, inquiries should be directed in every case to facts relating to these several causes.

Long-continued violent muscular exertions are supposed to lead to functional disorders of the heart. DaCosta has described cases occurring among soldiers during the late Civil War in which the cardiac disorder seemed to him referable to severe marches. He applied the name irritable heart to the condition in these cases.[4] It is probable that mental excitement had more or less to do with the causation. Albutt, Seitz, and other observers have attributed functional disorders to over-straining of the heart by occupations which call for severe exercise of the muscles.

[Footnote 4: Medical Memoirs of the United States Sanitary Commission, 1867. See Address before the Philadelphia Medical Society, by A. Stillé, 1883, p. 18. See also Diseases of the Heart among Soldiers, by A. B. R. Myers, London, 1870.]

{753} Paroxysmal disorder of the heart belongs among the multifarious symptoms referable to the nervous system in cases of hysteria. It is among the toxical manifestations embraced in the clinical history of gout, being referable, when it occurs in this pathological connection, to uricæmia. It may have this causation in cases in which the ordinary gouty manifestations do not occur.

In the variety of disorder characterized by infrequency of the heart's action it may be assumed that the causative agency is exerted through the pneumogastrics. The inhibitory function of this nerve is affected in the same way as by the galvanic current in the experimental observations on animals in illustration of this function. This view is corroborated by the frequent association of this variety of disorder with notable cerebral disturbance.

PROGNOSIS.--A purely functional disorder of the heart's action may be said to be devoid of danger to life. This is a remarkable fact, taking into view the importance of the organ, together with the degree and the duration of disordered action in some cases. Of many thousand cases which have come under my observation, I am not aware of having met with a single instance in which death was fairly attributable to an uncomplicated functional disorder. It is readily understood that functional disorders superadded to, albeit not dependent upon, organic affections of the heart may contribute to a fatal termination. But the tolerance of functional disorders under these circumstances is often very remarkable.

The assurance of the absence of all danger frequently lifts from the minds of patients a heavy load of anxiety and apprehension. To be able to give such an assurance is one of the delights of medical practice. Patients often find it difficult to believe that the disorder from which they suffer can take place while the heart is organically sound. Many require very positive and repeated assurances in order to secure their belief. The question is many times asked, "How is it possible that I should suffer so much, and yet the heart be free from disease?" Another question which is apt to be asked is, "How can you ascertain so quickly that there is no disease?" In anticipation of the latter question, in order to ensure the desirable moral effect, it is sometimes good policy to prolong the examination, inasmuch as for the exclusion of all the physical signs of organic disease a few moments only are required. Another question, still, is, "Will not organic disease be likely to be produced by the functional disorders?" The physician is fully warranted in giving a negative answer. Exclusive of the cases of exophthalmic goitre, functional disorders of the heart do not involve liability to either inflammatory or structural affections.

Recurrences of functional disorders of the heart constitute the rule rather than the exception. Their frequency will depend much on the degree of the predisposition, but of course more or less on the causes therewith associated. The mental anxiety and apprehensions which they at first occasion after a time wear away, and they are at length reckoned as belonging among those annoyances of life to which may be applied the common expression, "What cannot be cured must be endured."

TREATMENT.--Prompt relief or palliation of suffering is often the immediate object of treatment when cases first come under observation. The medicinal remedies for this object are the ethereal or alcoholic stimulants, the different antispasmodics, and opium. Chloric ether and the compound spirit of ether (Hoffman's anodyne) often act efficiently. An eligible prescription is the combination of one of these with an equal part of the compound tincture of lavender, of which a teaspoonful, properly diluted, may be given after short intervals. Brandy, whiskey, or some other form of spirit in many cases will afford prompt relief. It should be given not much diluted. These remedies {754} are especially indicated in paroxysms of irregular or intermittent and enfeebled action of the heart. They are less adapted to cases in which the heart's action is violent. Of antispasmodics, valerian, the valerianate of ammonia, camphor, and asafoetida are appropriate. Some one of the preparations of opium is to be employed if the disorder be not relieved by other remedies. Of the different forms of opiate, codeia is the least objectionable, and perhaps as efficient as any other. With a view to promptness of relief in certain cases of severity, morphia may be administered hypodermically. Other palliative measures are a sinapism to the præcordia, and, if the extremities be cold, a mustard pediluvium. Of the efficacy of the ice-bag applied over the heart, which is recommended by German writers, I cannot speak from personal observation. The testimony in behalf of its usefulness is, to say the least, sufficient for resorting to it without apprehension of doing harm. In some cases of obstinate persistence of disorder the opportunity is afforded for trying in succession the various remedies which have been named. Digitalis is sometimes useful. Concomitant disorders which may have originated or which tend to keep up the disordered action of the heart are to be appropriately treated. Flatulence and other ailments referable to indigestion and constipation not infrequently are in this category. Paroxysms may be sometimes arrested by certain mechanical means, such as pressure upon the abdomen, holding the breath after a deep inspiration, and compression of the vagus and sympathetic nerves in the neck.

In some cases of functional disorder there is a persistent increase of the frequency of the heart's action without irregularity in rhythm. The action of the heart in these cases is the same as in cases of exophthalmic goitre, the enlargement of the thyroid body and the prominence of the eyeballs which characterize the latter affection being wanting. In these cases aconite in small doses is to be recommended. From one minim to three minims of the tincture of the root may be given, repeated after intervals of four or six hours and continued indefinitely. In cases the opposite to the foregoing--namely, those in which the disorder is characterized by infrequency of the heart's action--a rational indication is to give remedies with a view to excite the heart. In the cases which have come under my observation alcoholics have had but little effect upon the heart, although apparently useful as regards the nervous symptoms which are apt to accompany this variety of cardiac disorder. As this disorder does not, as a rule, occasion distress, the patient perhaps not being conscious of any disturbances of the heart's action, and as the infrequency does not appear to involve danger, the treatment may be directed to fulfilling other symptomatic indications.

Positive assurances of the absence of danger have often a potential influence in relieving paroxysms of functional disorder. The disorder is not infrequently increased and kept up by mental apprehension, and these assurances therefore do away with an active causative agency. They are also useful in the way of preventing the recurrence of paroxysms. It is evident that in order to exert this moral influence the physician must be competent to decide that the disorder is purely functional. He can so decide only if he have confidence in his ability to exclude inflammatory and structural affections or to determine that the disorder is not dependent on lesions which may coexist. If he have not sufficient confidence in his opinion, he will naturally and properly not give positive assurances, and a lack of positiveness will be likely to lead the patient to infer that the disorder is not devoid of danger. The good effect of certain measures of treatment is in part attributable to a mental influence. This is legitimately a therapeutic object here as in other affections.

The more important part of the treatment in the majority of the cases of functional disorders of the heart's action is that which relates to prevention. {755} The preventive treatment, in addition to the moral influences already referred to, consists chiefly in removing as far as practicable the causes of the disorder. The predisposition cannot be removed, but the causes which are auxiliary thereto in producing disorder are, to a greater or less extent, controllable.

Prolonged mental anxiety is often inseparable from the events of life. "Therein the patient must minister unto himself" The voluntary exercise of the mental faculties, however, can be restrained within physiological limits. Tobacco can be abstained from, and, as a rule, total abstinence is easier than moderate indulgence. Tea and coffee can be used moderately if at all. Dyspeptic ailments are amenable to appropriate dietetic and medicinal treatment. On no account should the diet be reduced below the requirements for ample nutrition. Anæmia, which exists in a large proportion of cases, especially in women, calls for chalybeate tonics, to be continued persistently as long as the blood remains impoverished. It is needless to add that in these cases the causes of the anæmia are, if possible, to be removed, and that chalybeates are to be supplemented by proper dietetic and regiminal treatment. Sexual excess and abuses are to receive adequate attention. There can be no question as to unnatural sexual excitation. But a question often arises in individual cases concerning the physiological limitations of natural indulgence. These limitations probably differ widely in different persons. They are, however, always exceeded if the indulgence exceed the instinctive demand--that is, if its increase be made an object for voluntary efforts. Long-continued and violent muscular exertions should be interdicted. Uricæmia or the gouty diathesis claims appropriate remedies and hygienic regulations.

Several of the various causes just recapitulated are frequently combined, so that the preventive treatment is by no means always limited to the removal of a single cause. The treatment will prove successful in proportion as the efforts to remove the causes are effectual.

Angina Pectoris.

The name angina pectoris was introduced by Heberden in 1768 to designate a group of symptoms which from that date has been regarded as constituting an individual affection. The word angina, signifying strangulation, has but little pertinency in this application of it, and various other names have been proposed in its stead. For the most part these are based on pathological views which are either erroneous or hypothetical, and at the present time the name angina pectoris is generally adopted in all countries.

The affection may be defined as a paroxysmal neuralgia, the pain of which is seated within or near the præcordia, shooting thence in most cases into the left shoulder, and extending downward to a greater or less extent into the left upper extremity, the right upper extremity being sometimes similarly affected. In some instances the pain extends to the lower limbs; the paroxysms often accompanied by a feeling of anguish and of impending death, the affection in the great majority of cases being incident to organic disease of either the heart or the aorta and involving liability to sudden death.

SYMPTOMATOLOGY.--The foregoing definition embraces the prominent traits of a severe paroxysm. The pain may extend into situations other than those mentioned--namely, in different directions throughout the chest, into the neck, the jaws, and the temples, the abdomen, and the groin. In describing the pain patients use such terms as constricting, tearing, burning, etc. Perhaps in its most severe form there is no disease attended with more intense suffering. It is related that the description of the affection by Heberden led to a communication to him from an unknown correspondent who gave an account of his own case, and bequeathed to Heberden his body to be {756} examined after death. The examination was made by John Hunter, who himself fell a victim to the affection. An analogous instance occurred in my own experience. A patient was led by the intensity of his sufferings to request that I should make a post-mortem examination in his case, with the hope that something might be thereby ascertained which would prove useful to others. This request was complied with. Associated with the pain in severe paroxysms is what has been called a breast-pang, giving rise to a sensation as if death were at hand. A choking sensation, which is implied in the name angina, is an occasional symptom, resembling the globus hystericus. Respiration is not obstructed, but the patient may voluntarily restrain the respiratory movements lest they increase the suffering. Dyspnoea, if present, is thereby produced. During the continuance of the paroxysm the patient refrains from movements of the body or limbs, keeping a fixed position and grasping some firm support in order better to remain motionless while the pain lasts. A sensation of numbness in the affected limbs accompanies the pain. The circulation is usually more or less disturbed. There is sometimes increased and sometimes diminished frequency of the pulse. The action of the heart is often intermittent and otherwise irregular. It may be strong, but oftener it is weak. At the beginning the arterial tension has been found to be increased, but later is diminished. The face is generally pallid, but sometimes livid. The disturbances of the circulation are often modified by coexisting organic disease of the heart, but superadded are those of functional disorder incident to the paroxysm. The countenance is haggard and anxious. The surface of the body is cold, and may be bathed in perspiration. The mind remains unaffected. The paroxysms usually commence suddenly, and, as a rule, so end. Eructations of gas are apt to follow their cessation, together with a free discharge of limpid urine. The duration of a paroxysm may be but a few seconds; it is rarely longer than a few minutes. When it appears to be protracted for a considerable period, there is generally a series of attacks occurring in quick succession, instead of one continuous paroxysm.

There is much variation in different cases as regards the severity of the paroxysms, and the mildest offer a striking contrast to the severest, the essential symptomatic characters of the affection, however, being preserved. In mild paroxysms the pain is comparatively slight, the anguish or heart-pang is less, and the heart's action may be but little or not at all disturbed. Such paroxysms occasion annoyance without great suffering. Different cases, and the same case at different times, exemplify varying degrees of severity.

Recurrences of angina take place as a rule, to which there are but few exceptions. The intervals between the paroxysms vary in different cases, and often in the same case. Their recurrence is not governed by any law of periodicity. Generally, they are at first infrequent, and their frequency increases slowly. With increase in frequency their severity is apt to be increased. At first, and for a certain length of time, they are occasioned by some apparent exciting cause. A common cause is the exertion of walking, especially against a current of wind. Often for a considerable period patients are exempt whenever they are at rest. Sooner or later, in most cases, attacks are produced by other causes, such as a fit of anger or other mental emotion, and finally without any appreciable existing cause. I have known attacks to be caused by the act of swallowing solid food, so that eating became a source of terror to the patient. They occur in some cases during sleep. Occurring after intervals of a few moments, the affection in this respect resembling certain cases of tic douloureux, it doubtless would be difficult by any description to convey an adequate idea of the lamentable condition of the patient.

On account of the wide range of the gradations as regards the degree of severity or mildness, of the diversity of symptoms referable to the different {757} forms of disease of the heart with which the affection may be associated, and of the varied disorders which may be accidentally connected, the clinical picture of angina is by no means uniform. There is, however, no practical advantage in making formal varieties of the affection. Eulenberg makes four different types, their differential characters being based on the different nerves supposed to be especially affected, as follows: 1st, excito-motor cardiac angina; 2d, regulator angina; 3d, excito-motor sympathetic angina; and 4th, vasomotor angina. Assuming that there is ground for these pathological distinctions (which, to say the least, admits of doubt), in a practical point of view they involve difficulties not compensated for by important bearings on diagnosis and treatment. One point of distinction, however, has important bearings--namely, the existence of angina with or without organic disease of the heart. It cannot be doubted that in the vast majority of cases angina is incident to some form of cardiac lesion. That it may exist without any appreciable lesion is admitted. The propriety of recognizing it as a functional disorder rests on the latter fact.[5] Practically, the coexistence of organic disease of the heart or otherwise, and, if organic disease exist, its nature and extent, are points which it is important to take into account in the diagnosis with reference to prognosis and treatment.

[Footnote 5: Of 71 cases analyzed by Gauthier, in 3 only was the affection to be regarded as purely functional. Vide Eichhorst.]

DIAGNOSIS.--The diagnostic points in cases of angina are the præcordial seat of the pain, its radiations thence into the shoulder and upper extremity, generally of the left side, the character of the pain, the accompanying anguish and sense of impending death, the coexisting disorder of the heart (which occurs as the rule), and the voluntary immobility of the body. These are positive criteria which, if marked, render the diagnosis easy and certain. The diagnosis is further substantiated by finding the signs of organic disease of the heart, especially if there be lesions at the aortic orifice or within the aorta. Well-marked angina is in itself strong presumptive evidence of organic disease of the heart. Not infrequently the existence of the latter is for the first time discovered by an examination suggested by the occurrence of an attack of angina. The cases in which the diagnosis involves difficulty are those in which certain of the above-mentioned diagnostic points are either wanting or not well marked.

The affections which may be mistaken for angina are gastralgia and intercostal neuralgia. In gastralgia the pain is seated below the præcordia. It may radiate in different directions, but does not extend to the upper extremities, and is not accompanied by irregularity of the heart's action. The patient writhes and changes the position of the body in the effort to obtain relief. There is not a sense of impending death. The paroxysms are of much longer duration than those of angina. These differential points should suffice for the discrimination.

An acute attack of intercostal neuralgia does not differ so widely from angina, but the differential points are generally distinctive enough for a positive diagnosis. The pain in intercostal neuralgia is not seated in the præcordia. It does not shoot into the upper extremities; it is increased by the act of inspiration; the peculiar anguish of angina is wanting; the action of the heart is likely to be regular; and the diagnosis is confirmed by finding tenderness over circumscribed areas in the intercostal spaces anteriorly, laterally, and posteriorly.

Cardiac lesions in cases of angina are to be excluded by finding no physical signs of their existence. But it is to be remembered that angina is not infrequently associated with lesions not readily recognized by signs--to wit, obstruction of the coronary arteries and fatty degeneration of the heart. Persistent feebleness of the heart's action and symptoms other than angina {758} incidental thereto render it probable that one or the other or both of these lesions exist. It is probable that these lesions have been overlooked in examinations after death in some of the cases in which angina has been reported as not connected with any organic affection of the heart.

PATHOLOGY AND ETIOLOGY.--The paroxysms of angina have the distinctive traits of neuralgic affections as regards the character of the pain, its extension in the course of sensory nerves, the occurrence of intermissions, the absence of fever, the functions of digestion and assimilation remaining often unaffected, and the attacks not always being referable to any exciting cause. The association of the affection, as a rule, with organic disease of the heart is evidence of course of some pathological connection. What is this connection? A difficulty in answering this question arises from the fact that the affection is associated not with any one lesion, but with different lesions. It may be associated with obstruction (usually from calcification) of the coronary arteries, with insufficiency of the aortic valves, with rigidity from calcareous degeneration of the aorta, with aortic aneurism, and with fatty degeneration of the heart, these different morbid changes existing either singly or more or less of them in combination. The question then resolves itself into another--namely, What is the pathological condition common to these different lesions which stands in a special etiological relation to angina? It is a logical conclusion that the affection must depend upon some condition which is common to these lesions. The association with the lesions is too frequent to be explained by mere coincidence. The etiological relation involves evidently a condition which exists only in a small proportion of the cases of these lesions. This statement is a logical deduction from the great infrequency of angina and the frequency of these varieties of organic disease of the heart. I submit, as the most rational theory, that the pathological condition on which the angina depends is ischæmia of the heart. This theory is supported by the frequency of the instances in which in cases of angina the coronary arteries are obstructed; by the fact that not very infrequently this is the only lesion found after death (two instances having fallen under my own observation within the past year); by the association with aortic insufficiency and rigidity of the aorta, lesions which interfere materially with the supply of blood to the heart if it be admitted that the blood is driven into the coronary arteries, not during the ventricular systole, but by the recoil of the arterial coats in the ventricular diastole; and by the association with fatty degeneration of the heart when, owing to the weakness of the heart's action, the supply of blood to the muscular structure of the heart must be diminished. That the sudden withdrawal of a supply of blood to a part may occasion neuralgia is shown by the intense pain in the limb which directly follows embolism of the femoral artery. Moreover, general anæmia, as is well known, favors the recurrence of neuralgia in various situations.

The cardiac nerves in which the pain is seated are doubtless sensory fibres of the pneumogastrics. Their anatomical connections with the brachial plexus will explain the extension of pain to the left upper extremities. To account for the pain in parts which have no direct connection with the cardiac nerves, it may be assumed that in angina, as in other neuralgic affections, a centripetal influence conveyed to the nervous centres may occasion pain referable to different situations. This explains the shifting of pains which is one of the diagnostic traits of neuralgia. The explanation of the disturbed rhythm of the heart's action so often coexisting with the neuralgic pain is not more difficult than in cases of functional disorder disconnected from angina. For what is to be said of the rationale the reader is referred to that portion of this article which treats of Functional Disorders of the Heart.

{759} Angina, as a purely functional affection--that is, not symptomatic of any organic lesion of the heart, and not due to any structural change in, nor mechanical pressure upon, nerves--is obscure as regards its pathology and etiology, but not more so than many other neuralgic affections. As already stated, cases in which it is thus purely functional are few in number--fewer even than has been supposed, because there is reason to believe that lesions have been overlooked. Moreover, cases which have been reported render it probable that in some instances in which the heart has been found free from appreciable lesions nerves entering into the cardiac plexuses may be the seat of structural changes or may be subject to pressure from a morbid growth. But there are cases in which no lesions are discernible during life, and in which the existence of lesions is disproved by complete recovery. The affection under these circumstances must be regarded as purely functional. There is no positive knowledge of the etiology in these cases. The affection has been attributed to gout, to hysteria, to the action of cold, to the use of tobacco, and to other causes. These causes may have a certain amount of agency, but there is an unknown intervening link in their etiological connection concerning which, in the present state of our knowledge, it is useless to speculate.

Age and sex have an undoubted influence in the etiology. The affection very rarely occurs under middle life, and it occurs in men much oftener than in women.

PROGNOSIS.--As a very rare exception to the rule, a single paroxysm only may occur, the patient living for many years without any recurrence. Recurring paroxysms sometimes are separated by long intervals--weeks, months, and years. In the majority of cases, however, paroxysms recur with more and more frequency and with increasing severity. Under these circumstances death may take place after a long period of suffering.

The liability to sudden death is an important point in the prognosis. This may occur in the first paroxysm. An instance has fallen under my observation within a few months, there having been no signs previously indicative of disease of the heart. Calcareous obstruction of the coronary arteries was the lesion found after death. A person subject to paroxysms of angina must be considered as in more or less danger of sudden death with the recurrence of each paroxysm. The physician should be sufficiently impressed with the importance of this fact. While it is doubtful whether it be the physician's duty to apprise the patient of the fact, the danger should always be communicated to some discreet relative or friend. To do this is a duty which the physician owes to himself as well as to the patient. If he omit it, he exposes himself to censure should sudden death unexpectedly take place. The mildness of the paroxysms which have already occurred does not afford a positive security against the liability to a severe and fatal paroxysm. But it is a hopeful consideration that paroxysms may recur more or less frequently for an indefinite period without proving fatal. At this time I am cognizant of three cases in which paroxysms have recurred frequently for several years, the patients, with that exception, having had fair health. Let not the physician, therefore, predict with positiveness that a patient with angina will die sooner or later in a paroxysm. The uncertainty is a ground of encouragement as well as for apprehension.

The coexistence of organic disease of the heart and the nature of the cardiac lesions have a very important bearing on the prognosis. The danger is in proportion to the importance of these. Recovery is never to be expected when the affection is associated with well-marked cardiac lesions, and there is always great danger in the recurrence of paroxysms when the associated lesions are in themselves dangerous. Lesions which give rise to free aortic regurgitation and to fatty degeneration of the heart involve more or less {760} danger of sudden death, irrespective of angina. It is evidence of greatly increased danger if paroxysm of angina be superadded.

During a paroxysm of angina the immediate danger is to be estimated by the symptoms denoting disturbance of the heart's action. The danger is great in proportion as the action of the heart is feeble, irregular, or intermitting. Per contra, the danger is less in proportion as the deviation from the normal force and rhythm is small. It may be said that there is no danger so long as the heart's action remains unaffected, but the disturbance may be slight or wanting at the outset of a paroxysm and afterward become fatally great.

A favorable prognosis may be entertained when there are no signs of cardiac lesion, and when there is little or no disturbance of the heart's action during the paroxysms. Let it be borne in mind that such cases are exceptional and extremely rare. Let it also be borne in mind that lesions especially apt to be associated with fatal paroxysms may be latent--namely, obstruction of the coronary arteries and fatty degeneration. The latter fact renders it proper that a favorable prognosis should always be formed with a reservation, while the fact that recovery takes place in a few well-marked cases of angina renders it improper to withhold encouragement whenever lesions are not discoverable and the paroxysms are not accompanied by alarming symptoms referable to the heart's action. The long tolerance of the affection in some cases is not to be lost sight of with reference to the encouragement which may be fairly derived therefrom.

The immediate cause of sudden death in a paroxysm is probably an arrest of the heart's action in diastole, or such a degree of diminution of the force of its action that the accumulation of blood within its cavities induces paralysis from distension.

TREATMENT.--It is important that a paroxysm of angina be treated as soon as possible, not alone with a view to the relief of pain, but to remove immediate danger. If the physician be present, an opiate in a form to act promptly should be given either by the mouth or hypodermically; the latter mode is to be preferred. Laudanum or a solution of a salt of morphia is the most eligible form if given by the mouth. If the heart's action be weak and irregular, a diffusible stimulant is indicated. If at once available, chloric ether, Hoffman's anodyne, and the compound tincture of lavender act efficiently. If these be not at hand, an alcoholic stimulant should be given, diluted but little, and the doses repeated at short intervals until the paroxysm ends and the disturbed action of the heart has ceased. The duration of paroxysms is generally so short that a physician is rarely present unless they recur after brief intervals. A patient, therefore, subject to angina should be provided with remedies and instructions as to their use at the instant a paroxysm occurs. The amyl nitrite, first recommended in this affection by Brunton, is a remedy of signal benefit in some cases. From two to five minims may be inhaled at the commencement of the paroxysm. It is especially indicated when the characters of the pulse denote arterial tension. Caution is to be exercised in its use if there be notable weakness of the heart's action. Sinapisms, stimulating embrocations, and fomentations applied to the chest have a certain measure of utility, but they should not take the place nor delay the use of remedies which are more efficient.

A still more important object of treatment than relief in the paroxysms is their prevention. During the intervals this object claims assiduous attention. First in importance is the avoidance of all exciting causes. Bodily exercise is to be kept within the limits required in order to incur no risk of a paroxysm being produced. The same precaution applies to mental excitement. Unhappily, this is not as easy as the avoidance of muscular exertion. John Hunter's saying, that his life was at the mercy of any scoundrel who chose to {761} insult him, proved a prediction. He fell dead on receiving an insult from one of his colleagues at St. George's Hospital. Sexual intercourse I have known to prove an exciting cause. Excesses in eating and drinking are in this category. The diet, however, is not to be reduced below the full requirements for nutrition, and wine or spirits, as conducive to digestion, are in some cases serviceable. The use of tobacco is to be interdicted.

Coexisting affections which have no special pathological connection with the angina may act as auxiliary causes, and therefore claim attention. Gout is to receive appropriate treatment. Anæmia especially is to be removed. This condition strongly conduces to the development and the continuance of neuralgic affections. Chalybeate remedies and the dietetic treatment are called for if this condition coexist. It is a rational indication to supply the heart with good blood if it be true that angina depends on an ischæmic condition of this organ.

Associated cardiac lesions are to be treated according to symptomatic indications, as in cases in which angina does not occur. Digitalis may be used under the proper restrictions. I have known this remedy to prove highly useful in preventing the recurrence of paroxysms. Nux vomica is sometimes useful as a cardiac tonic.

Various drugs have had repute as empirical remedies. Of these may be mentioned the preparations of zinc, arsenic, the nitrate of silver, phosphorus, the bromine salts, the iodide of potassium, and quinine. There is no proof that these remedies have any special therapeutical effect in this affection, but that they are sometimes useful there is abundant testimony. Trial should be made of them, with proper care in their administration. Electricity in the form of the induced and of the constant current has been advocated as not only serviceable, but as effecting in some instances a permanent cure.[6] Beard and Rockwell have found general faradization useful in a few cases.[7]

[Footnote 6: Vide Eulenburg in Ziemssen's Cyclopædia, vol. xlv. p. 54.]

[Footnote 7: Vide Medical and Surgical Electricity.]

Exophthalmic Goitre (Graves' Disease; Basedow's Disease).

This affection is characterized by three striking symptomatic events--namely, persistent increase of the frequency of the heart's action, enlargement of the thyroid body, and protuberance of the eyeballs. The name exophthalmic goitre relates to the last two of these three events. It is defective, inasmuch as it does not include the increased frequency of the heart's action, which is the primary one of the three events, and the only one which is never wanting. As an individual affection it was first described by Graves in 1835, although cases in which these events were associated had been previously reported. Parry collected 7 cases in which the affection of the heart was associated with thyroid enlargement, and in 1 of these cases exophthalmia existed. An account of these cases was published in 1825. The name Graves' disease, proposed by Trousseau, has been adopted by French, English, and American writers. Basedow's disease is the name given to the affection by German writers. The affection was described by Basedow in 1840 under the name Glotz augenkrankheit.

There are cases in which one of the events in this symptomatic triad is wanting, the cases in other respects corresponding to the affection. The exophthalmia is the event oftenest wanting, the goitre, the functional disorder of the heart, and the associated phenomena being the same as if protuberance of the eyeballs coexisted. In some instances the goitre alone is wanting. The name exophthalmic goitre is not strictly applicable to these cases, but that the affection is essentially the same as when the three events {762} are present cannot be doubted. It is a chronic affection, being in the great majority of cases of long duration. Exceptionally, it is developed suddenly and disappears after a few days. In these cases the affection has been distinguished as acute, but its claim to be so called rests exclusively on the shortness of its duration.

SYMPTOMATOLOGY.--Of the three cardinal events, the increased frequency of the heart's action is the first in the order of time. This precedes the other events usually for several weeks or even months. The frequency varies in different cases within wide limits--namely, from 90 or 100 to 150 beats, and even more, per minute. There is notable variation at different times in the same case. Generally, the frequency is greatly increased by exercise and mental emotions. In other words, irritability of the heart is in most cases a marked feature. As a rule, there are none of the disturbances of action, in other respects than frequency, which are found in cases of functional disorder not associated with exophthalmic goitre. The action may be intermittent or in other respects irregular, but in most cases the rhythm is not disturbed. The patient is conscious of the heart's action, and is annoyed by it, especially under any excitement; but there is not that distressing sense of the disorder which is felt in the paroxysms of palpitation with irregularity of action considered in the first division of this article. At the outset and for a considerable period there are no signs of any organic disease of the heart, or if the latter be present the association is accidental; the disordered action, as far as it relates to the affection under consideration, is purely functional. At a later period there may be enlargement of the heart as a result of long-continued increased activity of function. From the first cardiac murmurs are generally present at the base and over the body of the heart. These are blood-murmurs due to coexisting anæmia.

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