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J Thorac Cardiovasc Surg 1997;114:392-403
© 1997 Mosby, Inc.

SURGERY FOR CONGENITAL HEART DISEASE

FUNCTIONAL OUTCOME AFTER THE FONTAN OPERATION: FACTORS INFLUENCING LATE MORBIDITY

Thomas L. Gentles , FRACPa*, Kimberlee Gauvreau , ScDa, John E. Mayer , Jr. , MDb, Steven B. Fishberger , MDa**, Janice Burnetta, Steven D. Colan , MDa, Jane W. Newburger , MD, MPHa, Gil Wernovsky , MDa***

K.G. was supported in part by the Kobren Fund.

Received for publication Nov. 5, 1996; revisions requested Jan. 22, 1997 revisions received June 2, 1997; accepted for publication June 2, 1997. Address for reprints: Jane Newburger, MD, Department of Cardiology, Children's Hospital, 300 Longwood Ave., Boston, MA 02115.

Abstract

Objectives: The purpose of this study was to describe the functional outcome of a large number of patients after modifications of the Fontan operation and to investigate perioperative risk factors that might influence late functional state. Methods: A comprehensive cross-sectional review of the first 500 patients undergoing a Fontan operation at our institution was undertaken. Those surviving with an intact Fontan circulation were reviewed by questionnaire to assess functional status and medication history. Medical records, chest roentgenograms, echocardiograms, cardiac catheterizations, and laboratory investigations were also reviewed to assess postoperative status. Results: Three hundred sixty-three long-term survivors with an intact Fontan circulation were identified during cross-sectional follow-up. Median age at operation was 5.0 years (range 0.4 to 31 years), and median follow-up was 5.4 years (range 1.7 to 20 years). Most patients (91.1%) were in New York Heart Association class I or II. In a multivariate model, poor (class III or IV) functional state was associated with longer duration of follow-up ( p < 0.001), a prior atrial septectomy ( p = 0.03), and a prior main pulmonary artery-ascending aorta anastomosis ( p = 0.05). Conclusions: A poor functional outcome is uncommon after the Fontan operation but becomes more frequent with increasing duration of follow-up.




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