[Pulmonary atresia with ventricular septal defect. Follow up and surgical results from 1963 to 1976 and diagnostic measures with special reference to large systemic-pulmonary collaterals (author's transl)].

Clinical course, surgical results, and diagnostic procedures of 76 patients with pulmonary atresia and ventricular septal defect between 1963 and 1976 were reviewed. 50 patients had bilateral intrapericardial pulmonary arteries with confluence (group A) and 4 patients had a unilateral intrapericardial pulmonary artery (group B). In 22 patients intrapericardial pulmonary arteries could not be demonstrated (group C). 11 of 20 patients in group A with no surgical intervention died at a mean age of 1.4 years, 9 of 30 children died after operation at a mean age of 3.4 years. 2 of 6 patients died after Rastelli procedure. The additive mortality of the palliative procedures in this group was 22%. There are no deaths after the two Rastelli procedures in group B. 81 collaterals originating from descending aorta and 8 from subclavian artery or brachiocephalic trunc which supplied the lung were outlined. 20 collaterals from descending aorta and 4 from subclavian arteries had connections with the intrapericardial pulmonary arteries. In 17 of the former 20 stenoses were demonstrated. Stenoses were also present in 37 of 61 collaterals without connections with the intrapericardial pulmonary arteries in patients belonging to group A, B and C.