[Fontan operation for tricuspid atresia with dysplasia of the right ventricular myocardium and absence of pulmonary valve].

We report a case of successful staged repair of tricuspid atresia with dysplasia of the right ventricular myocardium and absence of the pulmonary valve. The patient underwent the modified Blalock-Taussig shunt operation at 1 month the bidirectional Glenn procedure at 11 months, and the total cavopulmonary connection procedure using extracardiac graft at 2 years. It is important to prevent the left ventricular outflow tract obstruction (LVOTO) caused by the non-functioning right ventricle when the Fontan completion. We were safely able to disconnect the pulmonary trunk from the right ventricle using the transesophageal echocardiography to evaluate the LVOTO during operation.