Anomalous Inferior Vena Cava Draining into the Left Atrium Associated with Intact Interatrial Septum and Multiple Pulmonary Arteriovenous Fistulae

A 30-year-old woman with cyanotic heart disease due to anomalous drainage of the inferior vena cava into the left atrium in the presence of an intact interatrial septum is reported. The diagnosis was established during life and the defect was successfully corrected by open-heart surgery. The patient failed to survive due to associated multiple pulmonary arteriovenous fistulae. The embryology of the intracardiac defect, the pathophysiology of these fistulae, and the associated advanced pulmonary atherosclerosis are discussed.