Modified total cavopulmonary shunt as a staged Fontan operation.

The left superior vena cava became occluded in an infant with hypoplastic left heart syndrome. After a bidirectional Glenn procedure, he presented with severe oxygen desaturation and right ventricular dysfunction; the left superior vena cava drained into the inferior vena cava through collateral veins. As salvage therapy, we created a modified total cavopulmonary shunt using only autologous tissue in which the right hepatic vein and inferior vena cava drained into the pulmonary artery via a lateral tunnel in the right atrium. Immediately after surgery, his oxygen saturation increased and right ventricular function improved.