The Fontan procedure in adults

A retrospective clinical study was performed to document the course of adult patients undergoing the Fontan procedure.

Between 1982 and 1994, 21 adults aged 18 to 40 years (mean age, 27 +/- 7 years) underwent a Fontan procedure. Anatomic diagnosis was tricuspid atresia in 9, double-inlet left ventricle in 4, and various single ventricles in 8. Four underwent a right atria-right ventricle connection, 13 had a right atria-pulmonary artery connection, and 4 had a lateral-tunnel Fontan. Three of these 4 had a snare-adjustable atrial septal defect. Preoperative risk factors assessed were left ventricular end-diastolic pressure greater than 10 mm Hg, ejection fraction lower than 0.45, mean pulmonary artery pressure higher than 15 mm Hg, transpulmonary gradient greater than 10 mm Hg, pulmonary artery abnormalities, and atrioventricular valve regurgitation. Mean preoperative risk score was 1.6 /-1.1. Mean New York Heart Association class was 2.6 +/- 0.5.

The operative mortality rate was 5% (1/21). Six patients (30%) had a major complication, four being prolonged effusions. One patient was lost to follow-up; the remaining 20 have been followed for a mean of 7.4 +/- 3.8 years. At follow-up, mean New York Heart Association class was 1.7 +/- 0.5. There has been one late death (5%) at 9 2/3 years, which was probably due to ventricular arrhythmia. Three patients (16%) have required and survived reoperation. During follow-up, 7 patients (37%) have had development of atrial arrhythmias requiring medication, and 2 have been treated for ventricular arrhythmias.

These results indicate that properly selected adults can undergo the Fontan procedure with low morbidity and mortality. However, late-developing arrhythmias, need for reoperation, and decreasing ventricular function are serious problems that mandate careful follow-up.