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Circulation, Vol 85, 469-496, Copyright © 1992 by American Heart Association
DJ Driscoll, KP Offord, RH Feldt, HV Schaff, FJ Puga and GK Danielson
BACKGROUND. The purpose of this study was to estimate survival and quality
of outcome and assess factors associated with outcome for patients out 5 to
15 years from their Fontan operation. METHODS AND RESULTS. We studied 352
patients who had the Fontan operation prior to 1985. The overall 1-, 5-,
and 10-year survival was 77%, 70%, and 60%, respectively. The following
factors were significantly associated with lower survival: univentricular
heart or complex congenital anomalies other than tricuspid atresia, early
calendar year of operation, heterotaxia syndromes, early age at operation,
increased pulmonary artery pressure, atrioventricular valve dysfunction,
and higher (worse) New York Heart Association class. Reoperations were
necessary for 103 of the 352 patients. At least 20% of the survivors have
or have had cardiac arrhythmias requiring antiarrhythmic medication or
mechanical pacemaker insertion. Between 7% and 10% of the patients have had
or had protein-losing enteropathy/hypoproteinemia. At 5 years
postoperatively, 122 patients (34.7%) were alive with a better New York
Heart Association functional classification than preoperatively.
Fifty-eight patients (16.5%) were alive and in the same functional
classification, but 126 (35.8%) died within the first 5 years or were in a
worse functional classification. Thirty-nine patients were doing
excellently and 29 patients poorly 5 years after the operation. Of the
surviving patients, 43% can do as much exercise as their peers, whereas 3%
are incapable of exercise. CONCLUSIONS. To assure good functional long-term
outcome in addition to survival, clinicians must exclude from selection for
Fontan operation patients known to be at high risk for death or poor
outcome.
ARTICLES
Five- to fifteen-year follow-up after Fontan operation
Section of Pediatric Cardiology, Mayo Clinic, MN 55905.
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T.-Y. Hsia, S. Khambadkone, J. E. Deanfield, J. F. N. Taylor, F. Migliavacca, and M. R. de Leval Subdiaphragmatic venous hemodynamics in the Fontan circulation J. Thorac. Cardiovasc. Surg., March 1, 2001; 121(3): 436 - 447. [Abstract] [Full Text] [PDF]
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