Major intrahepatic venovenous fistulas after a modified Fontan operation

After the Fontan operation, systemic venous hypertension drives transpulmonary blood flow. Owing to this physiology, there is a significant incidence of effusions, splanchnic congestion, and generalized edema. To attenuate the effusive problems, partial exclusion of the hepatic veins has been recently practiced by Jacobs and Norwood. This report concerns a patient we recently treated for profound progressive cyanosis due to an acquired intrahepatic venovenous shunt after a Fontan operation with partial hepatic vein exclusion.