Successful staged Fontan completion for truncus arteriosus with hypoplastic left ventricle.

We report a case of truncus arteriosus type II with a large outlet ventricular septal defect and a hypoplastic left ventricle. The first-stage palliation was the removal of both branch pulmonary arteries from the ascending aorta, patch augmentation of the pulmonary artery confluence, and modified Blalock-Taussig shunt at age 1 month. The second stage palliation was a bidirectional Glenn at 6 months. The final stage was an extracardiac Fontan at age 3 years. Eight years later, the patient is doing well, with an unobstructed Fontan pathway and mild-to-moderate truncal valve insufficiency.