Two-dimensional echocardiographic diagnosis of double outlet left ventricle with subaortic ventricular septal defect, pulmonary stenosis, and a 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 solitus, d-loop ventricles, d-malposition of the great arteries (S, D, D), severe pulmonary stenosis, and a subaortic ventricular septal defect in which the left ventricle was also severely hypoplastic. The anatomic features were shown with 2-dimensional echocardiography, and the diagnosis was later confirmed at cardiac catheterization. Many morphologic variations of this malformation have been described but to the our knowledge DOLV with a hypoplastic left ventricle has not been reported before.