Left heart hypoplasia with associated anomalies.

The malformed heart to be described was removed at necropsy from a 9-day old Bantu infant, and sent to the Department of Anatomy for study. Unfortunately neither the clinical nor necropsy records could subsequently be traced and the only information available was that the cause of death was thought to be partial atelectasis. The main vessels branching off the arterial trunk were identified for us by the pathologist who performed the necropsy. The congenital anomalies present in this heart have not previously been reported as occurring together. Defective development of the entire left side of the heart is present resulting in a hypoplastic left atrium, grossly stenotic left atrio-ventricular (A-V) opening, rudimentary left ventricle, and hypoplastic aorta. Associated with the left heart hypoplasia are a common brachiocephalic trunk, a bicuspid right A-V valve, and defective septum secundum development.