The fontan procedure for tricuspid atresia.

A modified Fontan operation was performed on nine patients, ages 8--24 years (mean 14 years), with tricuspid atresia. The right atrium and right ventricle were connected with a valved conduit in five patients and directly in four. A porcine inferior vena caval valve was used in two patients. Prior operations had been performed in eight patients. There were no surgical deaths. Complications included pleural effusions, low-output syndrome, renal failure and atrioventricular block. There were two late deaths, only one of which was of cardiac origin. One patients required reoperation because of residual atrial septal defect at 1 month and again at 56 months for conduit stenosis. One patient required a pacemaker. Follow-up extends 6--65 months (mean 34 months). Survivors are clinically well and active, although four are taking medications. Postoperative cardiac catheterizations were done in six patients. Right atrial pressures were elevated in each, with no gradients across the conduit or pulmonary valve except in the one patient with conduit obstruction. Three patients had small residual ventricular septal defects and one a residual Blalock anastomosis. The Fontan procedure is an effective operation for tricuspid atresia. It may be further modified by establishing direct continuity between right atrium and right ventricle without a valve conduit in patients with a functioning pulmonary valve. The long-term ability of the right atrium to maintain adequate pulmonary flow and the effects of chronically elevated right atrial pressure on hepatic function and the cardiac conduction system must still be examined.