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The Journal of Thoracic and Cardiovascular Surgery, Vol 88, 939-951, Copyright © 1984 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
H Laks, JC Milliken, JK Perloff, WE Hellenbrand, BL George, A Chin, TG Di Sessa and RG Williams
From 1975 to the present, 45 patients have undergone modifications of the
Fontan procedure for complex congenital heart disease. There were 30 males
and 15 females ranging in age from 2 to 38 years (mean 13 years). Primary
diagnoses were tricuspid atresia in 19, univentricular heart in 24, and
pulmonary atresia with intact ventricular septum in two. Right
atrial-pulmonary arterial connections were performed in 32 patients, 11
with conduits (seven with valves and four without) and 21 by direct
anastomosis with patch augmentation. Right atrial-right ventricular
connections were made in 13 patients, six with valved conduits and seven
without conduits. Follow-up ranged from 0.1 to 9 years, with a mean of 2.3
years. There were three early deaths (less than 30 days) (7%) and two late
deaths (5%) in this series, all in patients with a univentricular heart.
The late deaths were both related to venous hypertension. A venous assist
device was used in eight patients in the immediate postoperative period and
was effective in improving cardiac output and reducing fluid accumulation.
Postoperative Doppler flow studies in 15 patients revealed biphasic
pulmonary artery flow in all without distinction between the type of
connection or the presence of a valve. Cardiac catheterization was
performed in 16 patients a mean of 14 months postoperatively (range 1 to 42
months) and revealed a reduced cardiac index at rest. Exercise testing in
eight patients demonstrated a marked rise in right atrial pressure with a
reduced rise in the cardiac index, even in those without functional
limitations. With a mean follow-up of 2.3 years, 78% of patients were in
New York Heart Association Class I, 17% in Class II, and 5% in Class III.
We conclude that the Fontan procedure is an excellent operation in
carefully selected patients with tricuspid atresia and other forms of
complex congenital heart disease.
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