Outcome of the Fontan procedure in patients with tricuspid atresia.

Between 1973 and 1983, 90 patients with tricuspid atresia underwent the Fontan operation at the Mayo Clinic. Operative mortality was 12% overall and decreased to 7% during the second 5 year period. At postoperative follow-up of 65 patients (1 to 10 years, mean 4), 50 (86%) of 58 present survivors were in excellent condition. There had been seven late deaths. Catheterization 0.5 to 8 years (mean 2.7) postoperatively in 25 patients disclosed a mean right atrial pressure of 14 mm Hg (range 6 to 21) and a mean cardiac index at rest of 3.0 (range 1.8 to 4.2). Mean left ventricular end-diastolic pressure was 8.5 mm Hg postoperatively compared with 16.5 mm Hg preoperatively; the decrease was secondary to a decreased ventricular volume load (mean volume load index 10.2 preoperatively, 3.0 postoperatively). We recommend that patients with tricuspid atresia have the Fontan operation by age 6 years to protect myocardial function.