Hypertrophy of the Heart, especially of the right divisions-Autopsy-Patescence of the foramen ovale-absence of the pulmonary artery-communication of the aorta with both ventricles. BY J. S. ROHRER, M. D., OF PHILADELPHIA. With remarks by the Editor. To Professor Dunglison:— Sir-In the course of the last week, I was requested by a medical friend to make an examination of a child, whose peculiarly blue appearance during life excited much attention. The lesions presented by examination have been deemed, by several anatomists to whom the heart has been exhibited, if not unique, at least as extremely curious and rare-I send you the case with some remarks for publication in your valuable journal, and would thank you to append to it your observations, with reference to similar cases. Very respectfully yours, Chestnut street, Philadelphia, 30th July, 1840. J. S. ROHRER. History-In presenting this case, I regret exceedingly that no correct account of the symptoms could be furnished by any of the physicians in attendance. The parents of the child being poor were imposed upon by every species of cupidity and empiricism. A great number of persons were consulted at different times, but no correct or satisfactory information could be obtained from any. My medical friend, who requested ine to make the autopsy, though highly intelligent, did not pay much attention to the case, and has but an indistinct recollection of the symptoms. He states that the child was subject to paroxysins of dyspnoea, and that the skin was perfectly blue. The following particulars were obtained from the mother of the child. It was a male infant, eleven months old-large and well formed at birth-po blueness was observed the first day, the room being rather dark. On the second day a blueness was perceived which gradually increased until the third day, when it could easily be observed all over the body. The child seemed hearty, with the exception of the blueness of the surface; sucked well; in two weeks after, it began to decline; did not improve any more, but wasted away gradually, and never appeared to increase much in size. It had frequent spells of screaming; took fourteen drops of laudanum at a dose before it was three months old, which was subsequently increased in quantity. The laudanum was not discontinued until a short time before its death. At six o'clock on the evening preceding its death, spasms occurred, which terminated its existence at one o'clock in the morning. Two weeks before its death vomiting supervened, which continued until a short time before its death. The heart beat violently during the whole period of life; so much so that the pulsation could be perceived through the clothes; a pulsation was felt like the beating of the heart below the stomach near the hips; it is not recollected on which side; the pulse was very fast; the child moaned much and appeared in constant distress; would frequently have attacks as if it was suffocating; cold water applied to the feet would bring on those paroxysms immediately; had a throbbing of the arteries of the neck; appetite very good during the whole period; would eat frequently so much as to cause vomiting; was intelligent for its age; could not stand upon its feet; had no strength in its limbs; in those paroxysms would hold its breath for a long time and turn up the eyes. Was blue at all times, but occasionally purple, the nails on the hands and feet like indigo; had no bowel complaint, nor fever, and sucked well until the last. The breathing was not very hard, except in the paroxysms, to which it was subject. As many as fifteen doctors were employed, the last of whom was a Homœopathist. Necroscopy. The heart at its base is six inches in circumference; its length from the apex to the semilunar valves of the aorta, two inches and a half. The right ventricle, at the point where the pulmonary artery should have originated, is half an inch in thickness, and the middle and lower portion of it is but one fourth of an inch. The left ventricle is uniformly one fourth of an inch in thickness. The right auricle is much distended, being about three times as large as the left auricle. The musculi pectinati are very strongly developed. The lungs are normal. The left auricle is unusually small, and has five pulmonary veins. The foramen ovale is deficient in several parts; the right auricle communicating with the left by different apertures. The valve of the foramen, for instance, is not closed, and is one sixth of an inch in diameter. At the upper and lateral portions of the foramen ovale, are two more foramina, on each side, both of which would admit a common quill. On each side of the eustachian valve are one or two orifices, the largest of which would admit a blow-pipe. The valvulæ tricuspides are of a scarlet colour, slightly tumefied, and cartilaginous. The capacity of the right ventricle is much increased, and about four times as large as the left ventricle. Whilst the right ventricle would hold an ounce of fluid, the left would not hold more than two drams. There is a deficiency in the upper part of the septum between the ventricles. A probe introduced into the aorta will pass with facility into each ventricle, and thus a communication is established between the two great cavities. This deficiency in the septum forms a foramen of half an inch in diameter, which is so placed that the communication between the right ventricle and aorta is more perfect than between that vessel and the left ventricle. The columna carnea of the right ventricle are greatly developed-thus corresponding with the superior developement of the musculi pectinati of the right auricle. The mitral valves are normal. The internal membrane of the left ventricle natural in thickness and slightly tinged with yellow. The pulmonary artery is much smaller than common, not exceeding one line in diameter, and does not penetrate to the cavity of the right ventricle, but is suddenly closed, ending in a pouch near the root of the aorta, where it should have originated, viz. at the anterior and left part of the basis of the right ventricle. As it proceeds obliquely upwards in its proper course, on the left side of the aorta, it divides into two branches, at the root of which, the ductus arteriosus is inserted. Thus there is no direct communication between the pulmonary artery and the cavity of the heart. The branches of the pulmonary artery are very small, and will with difficulty admit the end of a probe. The ductus arteriosus is one sixteenth of an inch in diameter. The aorta as it arises from the ventricle, being placed immediately over the septum between the ventricles, is full half an inch in diameter, and suddenly diminishes to the usual size where the arteria innominata is given off. The semilunar valves of the aorta are normal, closing entirely the aortic orifice. Remarks.-The anatomical lesions presented in the preceding case are very remarkable; the most striking of which is, that but one vessel, namely, the aorta, communicated directly with the cavities of the heart, whilst the pulmonary artery terminated in a cul de sac in the muscular tissue of the right ventricle. The question at once arises, by what means was the pulmonary circulation maintained? The present state of physiological science compels us to suppose that such a circulation is essential to life, and that in this instance it must have been effected through some unusual channel. May not the blood have been conveyed in a retrograde course from the aorta through the ductus arteriosus to the pulmonary artery and by this vessel to the lungs? The function of the right ventricle in this case being vicarious in a great degree to that of the left, caused great hypertrophy of the parietes and dilatation of the cavities of the right side of the heart and correspondent atrophy of the left. The aorta, it will be seen, was also much dilated. The question next arises, how did this phenomenon take place? It is plain that the blood being received on the right side of the heart, was forced through the foramen ovale into the left auricle, and through the tricuspid valves, into the right ventricle. The blood being thus propelled, the left ventricle received a portion of it from the left auricle through the mitral valves and the opening of the septum, whilst the greater portion passed into the left ventricle through the opening of the tricuspid valves.-The blood having thus arrived in both ventricles, the simultaneous contraction of which forced it at once into the aorta, their combined force acting upon that vessel gradually dilated it, in consequence of the impetus being spent on its parietes. It will be recollected that the dilatation did not extend beyond its great curvature. Upon consulting several authors I have not been able to find any case in which the entire communication of the pulmonary artery with the right cavities was wanting. Several cases are detailed, in which a similar lesion of the septum of the ventricles occurred, and some cases are narrated in which the orifice of the pulmonary artery was extremely contracted; but no instance is cited in which there was congenital absence of direct communication between the right ventricle and pulmonary artery. The learned editor of the Medical Intelligencer would confer a great obligation upon myself and the profession if he would have the kindness to communicate his observations on this subject. Remarks by the Editor.. The pathological appearances found on the dissection of those who have died of cyanosis or kyanosis, are diversified and curious. All permit the admixture of venous with arterial blood so as to give rise to the colour of the surface so suikingly exhibited in the case described by our correspondent. Gintrac,' who was professor of anatomy and physiology at Bordeaux, has collected the appearances presented on the dissection of fifty-three cases. In twenty-two of these, the aorta was found to arise from both ventricles. In thirty-three, the foramen ovale was open; in fourteen, the ductus arteriosus was wanting; in four, the heart was single, consisting of one auricle and one ventricle; in five, the ventricular septum was imperfect; in twenty-two, the pulmonary artery was contracted; in five, that vessel was obliterated; in one, the aorta was obliterated; and in four, the aorta arose from the right ventricle, the pulmonary artery from the left. The various malformations of the heart bave been well investigated by different pathological writers, and especially by Meckel, and Hope, and more recently still by Warnatz of Dresden-the last writer having described them in reference especially to kyanosis." The various malformations as given by Dr. Hope, are as follows: 1. The heart single, like that of a fish consisting of one auricle and one ventricle, from which a trunk originates that presently divides into pulmonary artery and aorta; 2. Two auricles and one ventricle. In one case the patient attained the age of 22. 3. The foramen ovale remaining open, which is the most common malformation. 4. The foramen ovale and ductusarteriosus both remaining open. 5. The foramen ovale and ductus arteriosus open and the pulmonary artery obliterated at its origin. In one case, the cavity of the right ventricle was nearly obliterated, and in two others the septum of the ventricles was perforated. To this division the case, described by Dr. Rohrer, would seem to belong. 6. Ventricular septum deficient; auricular imperfect. 7. Ventricular septum deficient at the aortic orifice, so as to leave a common opening into that vessel from both ventricles; this malformation being generally accompanied with contraction of the pulmonary artery, frequently with an open state of the foramen ovale, and occasionally with obliteration of the pulmonary artery and patescence of the ductus arteriosus. 8. Ventricular septum perforated towards the base, associated with contraction of the pulmonary artery and palescence of the base. 9. Foramen ovale open, and pulmonary artery arising from both ventricles, and giving off the descending aorta; whilst the ascending aorta arises in the usual way.. 10. Aorta arising from the right ventricle, and the pulmonary artery from the left; the foramen ovale and sometimes the ductus arteriosus remaining open. 11. The right auricle opening into the left ventricle instead of into the right; and the ventricles communicating by an aperture immediately below the aortic valves. The foramen ovale open. The heart in the case described by our correspondent appears to resemble that of the higher reptiles (höheres Reptilienherz, of Meckel) the ophidian, saurian, and chelonian, which consists of two auricles with a partitioned 'Observations et Recherches sur la Cyanose ou Maladie Bleue. Paris, 1824. 2 Ilandbuch der Pathologischen Anatomie, von Johann Friedr. Meckel, u. s. w. Band 1, S. 419. Leipz. 1812. Art. Kyanosis, in Encyclopäd. Wörterb. der Medicin. Wissensch. Band xx. S. 608. Berlin, 1839. 4 Treatise on the Diseases of the Heart and great vessels, and Mayo's Outlines of Human Pathology, reprinted in Dunglison's Medical Library. Philad. 1839. ventricle or a single ventricle. Meckel' refers to many such cases, described by Pulteney, Hunter, Sandifort, Nevin, Abernethy, Cruikshank, Prochaska, Caillot and Duret, Corvisart, &c. and Warnatz to others by Kreyssig, Hartmann, Tiedemann, Gintrac, Senac, Wolf, Lexis, Spittal, Bird, Beckhaus, Hunter, and others; but although these cases greatly resembled each other in the fact of a ready communication existing between the ventricles, they differed greatly in the details. In Lexis's case, the aorta arose from the ordinary place in the left ventricle, which was more than usually developed; at the same time, there was an opening through the septum ventriculorum in a straight line with the emhouchure of the aorta, which permitted a free communication between the right and the left ventricle, in such sort that one half the aorta might be considered to open into the right and the other into the left ventricle. pulmonary artery was greatly diminished in size. The In a case related by Bird, besides hypertrophy of the heart, a ready communication existed between the right ventricle and the aorta, at the place where the pulmonary artery ought to have been, and at the same time the carotid arose from the left ventricle. In another case, related by Beckhaus," of a "blue" child, but a slight trace existed of the septum ventriculorum; the valve of the foramen ovale, which was open, and the eustachian valve were present; and the ductus arteriosus was divided into two branches, one of which went to the right and the other to the left lung from the aorta; the pulmonary artery was wholly wanting. Generally, the pulmonary artery has been found of very small size, (Abernethy, Sandifort, Stenson, Hunter, Nevin, Caillot and Duret, Hope.) Duret and Caillot not only found the mouth of the pulmonary artery very narrow, and the coats thinner than usual, but the artery itself entirely impervious, and the ductus arteriosus wholly obliterated. Ramsbotham' found the pulmonary artery entirely wanting, its place appearing to have been supplied by the bronchial arteries. Romberg, in a case of cyanosis, found the aorta near the heart terminate in a cul-de-sac. It had received its blood through the ductus arteriosus from the pulmonary artery which was of unusual size. The child lived four days. It would appear, therefore, from all the pathological investigations which bave been instituted, that the main causes of cyanosis, or of the admixture of venous with arterial blood, are; first, the want of closure of the foramen ovale; secondly, the patescence of the ductus arteriosus beyond the proper period. Thirdly, the patescence of both the foramen and the duct beyond the normal time. Fourthly, an abnormous opening or openings in the septum between the ventricles, which have thus a free communication with each 5 De Deformationibus Cordis Congenitis, &c. Berol. 1825, cited by Warnatz. Meckel, and Warnatz Op. citat. 7 London Medical and Physical Journal, Jan. 1829. See, also, Müller, in Horn's Archiv. für 1822, Hft. 3. Ilanter, Medical Commen taries by Duncan, ix. 325. * Dissert. de Corde Vasisque Majoribus, &c. Berol. 1824. |