Background. Surgical approaches to single ventricle variants include staged
, fenestrated, and completed Fontan operations. This study compares outcome
s with these modifications of the Fontan operation at a single center.
Methods. Preoperative risk factors and operative results were analyzed by m
ultivariate techniques in 129 patients undergoing modified Fontan operation
s since March 1988.
Results. Overall early and late mortality was 5.4% and 0.8%, respectively.
Before 1993, completed Fontan operation using right atrial to pulmonary art
ery anastomosis without fenestration was performed in the majority of patie
nts (44 of 58; 76%). During this period, 10 of 17 patients at high risk had
completed Fontan with three takedowns. In 1994, the staged hemi-Fontan and
modified Fontan with a lateral tunnel anastomosis and with or without smal
l fenestration (2.5 to 4 mm) were introduced. The majority of patients at h
igh risk during this period underwent hemi-Fontan followed by fenestrated F
ontan with no takedowns. Late atrial dysrhythmias occurred in 6 patients (4
.7%), generally with larger fenestrations or right atrial to pulmonary anas
tomoses. Three patients (2.3%) had a stroke, 2 with large (greater than or
equal to 4 mm) fenestrations. Of 38 fenestrations, 32 (84%) closed spontane
ously by 1 year. No protein-losing enteropathy occurred. Most patients (118
of 121) were in New York Heart Association class I/II 4.5 years postoperat
ively. By multivariate analysis, only Down's syndrome (p < 0.001) predicted
early mortality, whereas both Down's syndrome and a systemic right ventric
le decreased late survival (p < 0.006).
Conclusions. Proper selection of patients for modifications of the Fontan p
rocedure resulted in excellent early and late survival with a low incidence
of atrial dysrhythmia and stroke. Midterm functional outcomes were excelle
nt. (C) 2000 by The Society of Thoracic Surgeons.