Congenital coronary arterio-ventricular fistulae, associated with absence of proximal coronary artery from aorta.

A male newborn expired 5 days after birth, because of multiple congenital cardiac anomalies. The postmortem examination demonstrated tricuspid stenosis, pulmonary atresia, hypoplastic right ventricle, persistent venous valve, and so on. In addition, he had coronary arterio-ventricular fistulous connection, associated with absence of the proximal connection of the coronary artery to the aorta or the pulmonary artery. As the result, the coronary blood was totally supplied via the right ventricular-coronary channels. Histologic examination revealed that these arterio-luminal channels were characterized by sinusoidal mural structure.