First Description of Coronary Artery Ostial Atresia With Fistulous Origin From a Normal Right Ventricle

Abstract

Anomalous origins of both the left and right coronary arteries are rare but have been well documented when both arteries arise from the pulmonary trunk (Angelini et al., Circulation 105:2449–2454, 2002). An anomalous coronary arterial origin from the pulmonary arteries usually involves the left coronary artery (ALCPA) and less frequently the right coronary artery (ARCPA). At least three cases have been reported in which the right coronary artery arose abnormally from the left ventricle (LV), but none have been reported in which both coronary arteries took their origin from the right ventricle (Ippisch and Kimball, J Am Soc Echocardiogr 23:222.e1–222.e2, 2010; Okuyama et al., Jpn Heart J 36:115–118, 1995; Culbertson et al., Pediatr Cardiol 16:73–75, 1995). Ostial atresia with anomalous origin of a coronary artery from the right ventricle has been described only in pulmonary atresia with an intact ventricular septum and a hypoplastic right ventricle. In this setting, atresia of both coronary ostia with right ventricular origin of both coronary arteries is a rare variant. This report presents a neonate in whom the entire coronary arterial system arose from the right ventricle via a single fistula with no other intracardiac defects. To the authors’ knowledge, this anomaly has not been described previously.