[Fontan procedure for DORV with mitral atresia and anomalous hepatic vein connection to the left atrium--advantage of leaving right to left shunt in situ].

A 5-year-old boy with double outlet right ventricle, mitral atresia, pulmonary stenosis and anomalous hepatic vein drainage into left atrium underwent Fontan procedure leaving hepatic venous-left atrial connection. Systemic venous-left atrial connection provided enough filling volume of the systemic ventricle despite of high pulmonary resistance. Extra cardiac shunt between the systemic vein and left atrium would be the second best in fontan operation for cases with high pulmonary resistance.