Results of right atrial to right ventricular and right atrial to pulmonary artery conduits for complex congenital heart disease.

The Fontan procedure was initially proposed for tricuspid atresia (TA). We describe our experience with modifications of this operation and its application to previously uncorrectable congenital lesions. From 1975 to 1979, 26 patients have undergone a Fontan-like procedure, at a mean age of 14 years (range: 3 months to 23 years). Nineteen patients had TA, one patient had mitral atresia, four patients had a univentricular heart, one patient had a "criss-cross" heart, and one patient had pulmonary atresia. Previous procedures included eight Glenn shunts, 18 systemic to pulmonary artery (PA) shunts, two PA bands, and two atrial septectomies. Thirteen patients underwent right atrial (RA) to PA connections, with a valved conduit in nine patients and without in four. Two patients had simultaneous Glenn shunts. Thirteen had RA to right ventricular (RV) conduits, with a valve in 12 patients and without in one. There were five early deaths (<30 days, 19%) and no significant difference between the RA to PA connection (four deaths of 13) versus the RA to RV connection (one death of 13). The mortality rate was higher in patients undergoing RA to PA connection without valved conduit (one death of nine with a valve, three deaths of four without, p < 0.05), while the Glenn shunt did not affect mortality (one death of seven with, three of six without, p < 0.1). There were no late deaths (mean follow-up: 24 months). Seven patients underwent recatheterization with a mean RA pressure of 14 mmHg (range: 9--25 mmHg). Thus, the "Fontan" procedure can be done with an acceptably low mortality with good functional results, both for TA and other complex lesions.