Background. A retrospective clinical study was performed to document t
he course of adult patients undergoing the Fontan procedure. Methods.
Between 1982 and 1994, 21 adults aged 18 to 40 years (mean age, 27 +/-
7 years) underwent a Fontan procedure. Anatomic diagnosis was tricusp
id atresia in 9, double-inlet left ventricle in 4, and various single
ventricles in 8. Four underwent a right atria-right ventricle connecti
on, 13 had a right atria-pulmonary artery connection, and 4 had a late
ral-tunnel Fontan. Three of these 4 had a snare-adjustable atrial sept
al defect. Preoperative risk factors assessed were left ventricular en
d-diastolic pressure greater than 10 mm Hg, ejection fraction lower th
an 0.45, mean pulmonary artery pressure higher than 15 mm Hg, transpul
monary gradient greater than 10 mm Hg, pulmonary artery abnormalities,
and atrioventricular valve regurgitation. Mean preoperative risk scor
e was 1.6 +/- 1.1. Mean New York Heart Association class was 2.6 +/- 0
.5. Results. The operative mortality rate was 5% (1/21). Six patients
(30%) had a major complication, four being prolonged effusions. One pa
tient 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 Associat
ion class was 1.7 +/- 0.5. There has been one late death (5%) at 92/3
years, which was probably due to ventricular arrhythmia. Three patient
s (16%) have required and survived reoperation. During follow-up, 7 pa
tients (37%) have had development of atrial arrhythmias requiring medi
cation, and 2 have been treated for ventricular arrhythmias. Conclusio
ns. These results indicate that properly selected adults can undergo t
he Fontan procedure with low morbidity and mortality. However, late-de
veloping arrhythmias, need for reoperation, and decreasing ventricular
function are serious problems that mandate careful follow-up. (C) 199
7 by The Society of Thoracic Surgeons.