Objective: The extracardiac Fontan procedure, as compared with classic atri
opulmonary connections, may have the potential for optimizing ventricular a
nd pulmonary vascular function by maximizing the laminar flow principle, by
the avoidance of intra-atrial suture lines and cardiac manipulation, and b
y minimizing cardiopulmonary bypass time. In this study the clinical result
s of this procedure are assessed. Methods: From January 1990 until January
1997, 45 patients (33 males and 12 females) with a median age of 4.0 years
(range 2.7-38 years) underwent an extracardiac Fontan procedure for univent
ricular physiology. The underlying diagnoses included tricuspid atresia (n
= 19), double-inlet left ventricle (n = 11), and complex anomalies (n = 15)
. Forty patients (89%) were in sinus rhythm. The median ventricular ejectio
n fraction was 60%. In 37 patients (82%) the procedure was staged. Results:
Median cardiopulmonary bypass time was 72 min, with a decrease to a median
time of 24 min in the last ten patients. Aortic cross-clamping was avoided
in 33 patients (73%). The intraoperative Fontan pressure and transpulmonar
y gradient were low: 13.6 +/- 3.2 and 8.5 +/- 3.9 mmHg, respectively. Trans
ient supraventricular tachyarrhythmias were observed in six patients (13%).
There was no early or late mortality. At a median follow-up of 64 months (
range 26-105 months), 39 patients (87%) were in NYHA class I, four (9%) wer
e in NYHA class II, and two (4%) were in class III. Forty patients (89%) re
mained in sinus rhythm. The median ventricular ejection fraction was 59%. T
he median arterial oxygen saturation raised from 82% preoperatively to 97%.
Functional class (P = 0.02), maintenance of sinus rhythm (P = 0.04), and p
reservation of ventricular function (P = 0.05) was superior in patients who
were appropriately staged. None of the patients had atrial thrombus, chron
ic pleural effusions, or protein losing enteropathy. Conclusions: In the ma
jority of patients, the extracardiac Fontan procedure, when performed as a
staged procedure, provides excellent early and midterm results in terms of
quality of life, maintenance of sinus rhythm, and preservation of ventricul
ar function. (C) 2000 Elsevier Science B.V. All rights reserved.