Double-Outlet Left Ventricle with Ebstein Anomaly in a Neonate with VACTERL Association

Double-outlet left ventricle is a very rare congenital cardiac malformation in which both the aorta and the pulmonary artery arise predominantly or exclusively from the morphologic left ventricle. We describe a neonate with esophageal atresia with trachea-esophageal fistula (so-called VACTERL association) and unusual cardiac anomalies including double-outlet left ventricle, subaortic ventricular septal defect, pulmonary stenosis, and Ebstein anomaly. Many morphologic variations of cardiac malformation have been described in patients with VACTERL association, but to the best of our knowledge, double-outlet left ventricle combined with Ebstein anomaly has never been reported.