Systemic-pulmonary arterial supply in pulmonary atresia with ventricular septal defect: postmortem angiograms and...

Postmortem angiographic and histologic studies of the pulmonary arterial circulation were performed in a patient with pulmonary atresia and a ventricular septal defect. While the left lung was supplied by a closing ductus arteriosus, the right lung was supplied by two systemic pulmonary arteries arising from the descending aorta. The examination disclosed that systemic pulmonary arteries lead into the pulmonary vascular bed and the capillaries of the alveolar walls. According to these observations, such collateral circulation is to be considered functional. The pulmonary vascular bed, supplied by the ductus arteriosus and the stenotic systemic pulmonary artery, showed a thin muscular layer in the small arteries and arterioles. On the contrary, medial hypertrophy and severe intimal proliferation were observed in the pulmonary segments perfused by the other large unobstructed systemic pulmonary artery, thus proving that asymmetric pulmonary vascular disease may complicate the natural history of this malformation.