MITRAL AND AORTIC ATRESIA ASSOCIATED WITH HYPOPLASTIC RIGHT LUNG, CROSSOVER SEGMENT OF RIGHT LOWER LOBE, AND ANOMALOUS SCIMITAR-LIKE RIGHT PULMONARY VENOUS CONNECTION WITH INFERIOR VENA-CAVA - CLINICAL, ANGIOCARDIOGRAPHIC, AND AUTOPSY FINDINGS IN A RARE CASE

A newborn female infant was found to have a unique and previously unre ported group of anomalies: (1) mitral and aortic atresia with a highly obstructive atrial septum; (2) hypoplasia of the right lung with a cr ossover segment involving the right lower lobe; (3) normally connected pulmonary veins, two from the left lung and one from the right; and ( 4) a large anomalous branch of the right pulmonary vein of scimitar co nfiguration that anastomosed with the normally connected right pulmona ry vein and with the inferior vena cava (IVC). The scimitar vein appea red obstructed at its junction with the right pulmonary vein and at it s junction with the inferior vena cava within the hepatic parenchyma. To our knowledge, this is the first report of a scimitar-like vein coe xisting with mitral and aortic atresia and connecting both with the ri ght pulmonary vein and with the inferior vena cava. The highly obstruc ted left atrium was partially decompressed by retrograde blood flow vi a the normally connected right pulmonary vein to the anomalous scimita r venous pathway and thence to the inferior vena cava via a pulmonary- to-IVC collateral vein.