Ligation of the left hepatic vein for dilated intrahepatic collaterals late after fontan operation.

A 17-year-old male presented with two episodes of syncope, cyanosis, and exercise intolerance 13 years after the Fontan operation. Echocardiography and magnetic resonance imaging showed dilated intrahepatic collaterals, which drained into the atrium through the left hepatic vein, and 24-hour Holter monitoring revealed sinus node dysfunction. We performed ligation of the left hepatic vein using intrahepatic collaterals as channels draining hepatic venous blood into the Fontan pathway, and implanted an epicardial dual chamber pacemaker. At one-year follow-up, the patient remained asymptomatic and his cardiac performance was much improved.