Cor biloculare

Cor biloculare

L-- Clinical Reports -.-.-..-._. - .-.__ -_-.__ --_--_.... --------- COR BILOCULARE CASE REPORT J. K. BE~IBENISTA, M.D. BUFFALO. N. Y. BILOCULARE ...

862KB Sizes 10 Downloads 224 Views

L--

Clinical Reports -.-.-..-._. - .-.__

-_-.__ --_--_.... ---------

COR BILOCULARE CASE REPORT

J. K. BE~IBENISTA, M.D. BUFFALO. N. Y. BILOCULARE is a rare C ORrecorded by, Wilson in 1798.

congenital cardiac anomaly and was first Abbott, in a comprehensive study of congenital heart lesions published in 1936, recorded only fourteen examples of this anomaly. Since then, five additional cases have been reported, two of which occurred in twins. Most subjects with this condition die in early infancy, although one patient lived to the age of 18 years. The sex distribution is about equal. There are no characteristic physical signs; no cardiac murmurs or thrills have been described. Cyanosis is the most prominent finding. Other anomalies may be associated with this condition, the commonest of which is a persistent truncus arteriosus. CASE REPORT On March 12, 1941, a white baby girl, weighing 8 pounds and 11 ouncc~,was born at the Mercy Hospital. The delivery was spontaneous from a vertex presentation under vinethene anesthesia. The mother was 36 years of age and has five other normal children. Her past history is not remarkable. The child’s father is living and well. Immediately after delivery the child’s color was good and it had a strong cry. About three hours later, it was observed that the baby became cyanotic when she cried. Examination of the infant at this time revealed no other abnormalities. No murmurs were heard over the precordium. An x-ray film showed no enlargement of the thymus gland. . For the first few days of life the baby’s color was fair until it sneezed or cried; then cyanosis became pronounced. Later cyanosis became constant and was not relieved even when oxygen was given continuously. The baby had several vomiting spells with emesis of blood-streaked mucus. Its condition became progressively worse, and it expired the seventh day after birth. Auto#y Findings.-External examination showed a well-developed and fairly well-nourished but cyanotic female infant; the only abnormality was a small angioma of the skin of the back. Examination of theinternal organs revealed no important findings except in the heart. When viewed in situ, the portion of the heart usually formed by the right auricle was seen to be slightly enlarged; the part of the left border of the heart usually formed by the left ventricle The appeared more globular than usual. The pulmonary artery was in its proper position. ductus arteriosus was present but somewhat narrow, especially in its mid-portion. Recehod for publication

October 13. 1946. 394

BEYBENISTA

:

COR BILOCULARR

395

On opening theheatt only one atria! chamber was found, from which two auticulat appendages projected and into which al! the veins entered. The common atrium communicated through a large aperture with a thick-walled ventricle, which in turn emptied into a normal-sized aorta. Near tlie upper margin of the ventricle and in its anterior wall lay a small, flat, slitlike cavity which evidently was a rudimentary tight ventricle. It communicated with the pulmonary artery by an opening guarded by three small but normal valve cusps. Although this chamber communicated with the large ventricle by a tiny orifice, it contained no blood and apparently did not function. This infant’s heart thus had only one atrium, one useful vcntticle, one rudimentary and nonfunctioning ventricle, and a small ductus arteriosus Blood entered the pulmonary artery only through this insufficrrnt ductus arteriosus. On mote detailed examination St was found that four large veins entered the atrium. On the right side superiorly was the opening of the superior vena cava. The posterior margin of thi* opening was formed by a small muscular bundle, fotming a semilunar ridge. This normally is the upper limbus of the fotamen ovale. Posterior to and just below the orifice of the superior vena cava, the right and left pulmonary veins entered. Below these lay the opening of the inferior vena cava. This opening was partly coveted by a fibtomusculnt membrane which most likely was the rudiment of the intetattia! septum. The opening of the coronary sulcus lay between that of the inferior vena cava and the atrioventrirular aperture.

Fig. 1.-The

single auricle and the only functioning ventricle are shown.

The attioventricular opening was 4 cm. in circumference and was guarded by four unequal valve leaflets, to which were attached four groups of papillary muscles. The ventricle was as large as the combined right and left ventricles of a normal heart at birth. The thickness of its muscle wall averaged 6 millimeters. From Its superior portion arose the aorta with a normal semilunar valve composed of three cusps. .Just below the base of the right posterior cusp of this valve was a tiny opening in the wall of the ventricle. This tiny orifice was the only communication between the large ventricle and the slitlike cavity of the rudimentary right ventricle previously described. The pulmonary artery was in its normal position and was guarded by three small cusps of the pulmonary valve The coronary arteries were not remarkable. The lung, liver, spleen, and kidneys showed passive congestion.

3%

AMERICAN‘

IIEART

JOURNAL,

SUI\I MARY

The heart described was unusual in that it consisted primarily of only one atrium and one ventricle with a patent ductus arteriosus. The ventricle had a rudimentary slitlike cavity in its wall, but this to all intents and purposes was nonfunctioning. This case, we feel, is a car biloculare.

REFERENCES

1. Tow, A.: Cor Biloculare With Truncus Arteriosus and Endocarditis, Am. J. Dis. Child. 42: 1413, 1931. 2. Abbott, -M. E.: Atlas of Congenital Cardiac Disease, New York, 1936, The American lieart Association. 3. Davies, F., and MacConaill, M. A.: Cor Biloculare W’th Note on Development of Pulmonary Veins, J. Anat. 71: 437, 1937. 4. Guistra, F. X., and Tosti, V. G.: True Cor Biloculare in Identical Twins, Ah!. HW.RT J. 17: 249, 1939. Commune 5. Benjamin, J. E., Landt! H., and Zeek, P.: Persistent Ostium Arterioventriculare in Heart Which FunctIoned as Biloculate Organ: Report of Case, Including Autopsy, in an Eighteen-Year-Old Girl, AM. HEART J. 19: 606, 1940. 6. Rossman, J. I.: Cor Biloculare With Transposition of Great Cardiac Vessels and Atresia of Pulmonary Artery, Am. J. Clin. Path. 12: 534, 1942. R. P.: Cor Biloculare With Persistent Truncus Arteriosus, ASP. IIEART J. 25: 7. f;:4h;iTjn, t *