Double-Outlet Right Ventricle with Transposed Great Arteries and Subpulmonary Ventricular Septal Defect

Abstract

The angiocardiographic findings and successful surgical treatment of three patients with double-outlet right ventricle, bilateral conus, transposition of the great arteries, subpulmonary ventricular septal defect, and pulmonary-mitral valve discontinuity are reported. The atria, viscera, and ventricles were normally located, and the aorta originated to the right and anterior to the pulmonary artery. Surgical repair was accomplished by closure of the ventricular septal defect in such a way that left ventricular outflow was routed via a prosthetic tunnel to the pulmonary artery. Then systemic venous return was transposed intra-atrially by the Mustard technique. All patients survived complete repair and have continued to improve over a period ranging from several months to 1 year after operation. In one patient complete heart block developed postoperatively, necessitating the insertion of a permanent electronic pacemaker. Details of the method of repair and preoperative studies are presented.