[A successful conversion of failed fontan circulation to total cavopulmonary connection in a case of tricuspid atresia without pulmonary stenosis].

A 12-year-old boy was first admitted to Tokyo Metropolitan Children's Hospital at 2 days of age with the diagnosis of tricuspid atresia without pulmonary stenosis, normally related great arteries, ventricular septal defect, and persistent left superior vena cava (PLSVC). He underwent pulmonary artery banding at the age of 1 year and atrial septectomy at 2.9 years. At the age of 6.3 years, he underwent successful Bjork operation in conjunction with closure of a trial and ventricular septal defects. Late after surgery, however, he developed progressive intolerance to exercise (NYHA III), cardiomegaly, and depressed ST segments in the left precordial leads. Catheter examination at the age of 11.9 years disclosed moderately elevated pressures (15 mmHg) in the right atrium and pulmonary artery, depressed left ventricular function with ejection fraction (EF) of 0.48, and marked dilatation of the coronary sinus (CS). Right atriography revealed retrograde filling of the dilated CS up to the junction with PLSVC. With supposition that the combined elevation of the volume and pressure loads on CS had disturbed both coronary circulation and ventricular function, he, at the age of 12 years, underwent take down of the Fontan circulation and conversion to the total cavopulmonary connection (TCPC). His postoperative recovery was uneventful and, at 2.5 years after the latest surgery, he is leading a normal life. The findings of the chest roentgenogram and electrocardiogram reverted to normal and EF returned to the normal value (0.66). This experience draws us to conclude that conversion to TCPC is a safe and effective mean to salvage the patients with failed Fontan circulation. This experience also suggests that dilatation of CS is a predictor of the failed or failing Fontan circulation.