Fontan procedure and surgical modification in tricuspid atresia.

Nineteen patients with tricuspid atresia and reduced lung perfusion (valvular-and/or subvalvular pulmonary stenosis, transposition of the great arteries and/or single atrium) were operated in the period 1975--1979. The surgical procedures employed varied according to the additional cardiac defects. The age of the patients was between 2 and 18 years. Eleven children received a contuit with a Hancock valve, 8 children a valveless conduit. In 9 patients it was possible to connect the conduit to the right ventricle using the pumping action of the right ventricle with an anatomically intact pulmonary valve. Six patients died (4 early deaths, 2 late deaths, early mortality rate 21%). In this study, the postoperative courses, which were complicated in several cases, are related to the hemodynamical findings. Thirteen children were examined between 1 and 38 months following the operation. Cardiac catheterization was performed in 10 patients. Of the 13, 10 had fully saturated arterial blood. A remnant atrial defect was demonstrated in one child, and 2 displayed intrapulmonary shunts attendant to Glenn anastomoses which had been in place for 10 to 12 years. Among the patients the right atrial pressure ranged from 10 to 20 mmHg with a mean value of 14.5 +/- 0.9 mmHg. An increase in size of the right ventricle was demonstrated angiographically in the case of 2 patients who had valve-bearing conduits to the right ventricle.