Double outlet left ventricle (DOLV) with ventricular septal defect (VSD)

Double-outlet left ventricle (DOLV) is a rare congenital cardiac malformation in which both great arteries originate entirely or predominantly from the morphologic left ventricle. DOLV occurs most commonly in the form of atrial situs solitus with atrioventricular (AV) concordance. Ventricular septal defect (VSD) is most commonly subaortic, and 90% of these patients have associated pulmonary stenosis. In most cases, there is a right anterior position of the aorta relative to the pulmonary artery. Most patients with DOLV will have pulmonary outflow obstruction and present soon after birth with cyanosis. DOLV with two well-developed ventricles is conventionally treated surgically by VSD closure, closure of the native left ventricle-to-pulmonary outflow, and placement of a right ventricle-to-pulmonary artery extracardiac conduit. However, pulmonary root translocation may be used as an alternative to biventricular repair of DOLV. Patients with DOLV and functionally single-ventricle or AV valve atresia will require a Fontan procedure.