ASSOCIATION OF CONGENITAL HEART DISEASE WITH DOWNS SYNDROME (MONGOLISM)

Between 1957 and 1964 in California there were 7,625 known deaths due to congenital heart disease; in 367 of these cases the subjects had Down's syndrome. Autopsy records from 14 hospital centers allowed more specific anatomic diagnoses in 139 of the 367 cases. Five types of defects, with some overlap, accounted for 91.4 per cent of the cases: ventricular septal defect in 55 cases (32.4 per cent), atrioventricularis communis in 34 (24.5 per cent), atrial septal defect in 28 (20.1 per cent), patent ductus arteriosus in 16 (11.5 per cent) and tetralogy of Fallot in 15 (10.8 per cent). It was not possible to ascertain how many of the atrial septal defects were of the ostium primum variety, nor how many of the ventricular septal defects were of the atrioventricular canal type. The 139 cases are compared with 172 documented anatomic findings from four other studies. The major difference in this, the largest single study, is the larger number with ventricular septal defect as opposed to atrioventricularis communis. Many of the ventricular septal defects were associated with other defects, however. © 1969.