Double-Outlet Left Ventricle with a Superiorly Positioned and Hypoplastic Left Ventricle

Double outlet left ventricle (DOLV) is a rare congenital cardiac malformation in which both the pulmonary artery and the aorta arise exclusively or predominantly from the morphologic left ventricle. We describe a case of DOLV with situs inversus, L-loop ventricles, d-malposition of the great arteries (I, L, D), superior–inferior ventricles, unbalanced complete atrioventricular septal defect, pulmonary stenosis, and total anomalous pulmonary venous return in which the left ventricle was also severely hypoplastic and superiorly positioned. Many morphologic variations of this malformation have been described, but to the best of our knowledge DOLV with superiorly positioned and hypoplastic left ventricle has not been reported before.