[Fontan procedure in patients with hypoplastic left heart syndrome].

In the recent years, the Fontan completion rate for hypoplastic left heart syndrome (HLHS) is improved because of the outcome of stage-1 palliation improved. However, there are still problems to be solved to achieve better hemodynamics after Fontan operation in patients with HLHS. The 2 major commandments other than classic 10 commandments are A:"good ventricular function" and B:"low pulmonary vascular resistance". To obtain these 2, the following problems should be rectified. They include residual arch obstruction, impaired right ventricule (RV) function, tricuspid regurgitation, elevated pulmonary vascular resistance, development of aorto-pulmonary collaterals and etc. The modern strategy for the treatment to achieve "good" Fontan circulation in HLHS patients, are 1)Norwood procedure with RV-pulmonary artery( PA) conduit, 2)staged palliation strategy, 3)repair co-existent and residual abnormality before stage-3(preferably at stage-2 palliation), 4)coil embolization of aorto-pulmonary collateral arteries before Fontan procedure, 5)the type of Fontan operation is extracardiac total cavopulmonary connection (TCPC) type, 6)fenestration if necessary, 7)careful and continuous follow-up is mandatory for early detection of arrhythmia, protein losing enteropathy (PLE), liver dysfunction and other fatal complications of long term after Fontan type procedure.