Surgical ligation of anomalous hepatic vein in a case of heterotaxy syndrome with massive intrahepatic shunting after modified fontan operation.

In case of progressive cyanosis after modified Fontan procedure, intrahepatic shunting should be considered. Ligation of the hepatic vein is safe and effective in managing this condition, and it is important to ensure that the pressure difference between superior vena cava and the portal vein system remains minimal after clamping of the hepatic vein before ligation is completed to minimize the possibility of varix due to portal vein hypertension.