Abstract
Ectopia cordis (E.c.) is a congenital malformation in which the heart lies partially or totally outside mediastinum. According to Shao-Tsu, 210 cases of E.c. had been reported up to 1957. In Japan, 13 cases of E.c. which include 6 cases of E.c. thoracalis, 5 cases of E.c. thoraco-abdominalis and 2 cases of E.c. abdominalis were found in the literature up to 1963. We added 3 cases of E.c, which is described below. Case 1. Male, B.W. 700 gm. The clinical history of the mother was unknown. heart: 1. E.c. cervicalis 11. Primitive heart with pulmonary atresia. a) Common (single) atrioventricuar arifice. b) Single atrium c) Membran formation of atrium. d)Single inflow tract. e) Single outflow tract. f) Hypoplasia of the pulmonary artery and ductus arteriosus. 111. Single coronary artery. 1V. G-type. Case 2. Male infant, B-W 1670 gm. The clinical history of the mother was unknown.. heart: I. E.c. thoracalis. 11. Riding aorta with pulmonary atresia. a) Atresia of the pulmonic valve. b) Hypoplasis of the pulmonary artery and ductus arteriosus. c) V.S.D. d) Dilatation and hypertrophy of the Right ventricle. e) Adnormal configuration of the C.S.V. 111. Persistent left superior vena cava drainaged into right atrium. a) Dilatation of Coronary sinus. b) Dilatation of right atrium. 1V. A.S.D. V. Partial anomalous pulmonary drainage. VI. Hypertrophy of the endocardium VII. pericarditis. VIII. Bloodcyst of the T.V. and M.V. Case 3. Male, B-W. 680 gm. There was an anencephalic monster on the mother's side. Only polyhydroamnios was known in the clinical history. Heart: I. E.c. thoraco-abdominalis II. Complete transposition of the aorta with mitral atresia. a) Common ventricle b) Hypoplasia of the aorta with fetal coarctation of the aorta. c) A.S.D. and fenestration. d) widely tricuspid valve e) Bicuspid aortic valve. f)Dilatation of right atrium. g) Hypoplasia of the left atrium. III Single coronary artery IV. Abnormal branches of the great vessel. All of the cases were male. They showed the different types of E.c. from each other and were all found to be accompanied with cardiovascular anomalies as above mentioned. Ectopia cordis frequently associated itself with cardiovascular anomalies. In the report of E.c., cardiovascular anomaly is found 52(72%) out of 72 cases discussing the morphology of the heart. The cardiovascular anomalies associated with B.c. are varied, but V.S.D. and Fallot's tetralogy are the most common types. Some scholars have noticed particulary liable to associate persistent left superior vena cava and E.c. Judging from these facts, there seems some organic relation between the morphogenesis of cardiovascular anomalies and E.C., regardless of the type of the former. Apparently, in all our cases, E.c. is related either directly (case 1 & 3) or indirectly (case 2) with amnions. It is a most difficulty to decide which of ectopia and the amnionic adhesion is primary. However in our three cases it is estimated that the abhesion and pluling of amnions in the earlier embryonic stage is primary, and that it gave rise to the hemodynamic change of heart, which in turn induced cardiovascular anomalies.
