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J Thorac Cardiovasc Surg 1997;114:376-391
© 1997 Mosby, Inc.

SURGERY FOR CONGENITAL HEART DISEASE

FONTAN OPERATION IN FIVE HUNDRED CONSECUTIVE PATIENTS: FACTORS INFLUENCING EARLY AND LATE OUTCOME

Thomas L. Gentles , FRACPa*, John E. Mayer, Jr. , MDb, Kimberlee Gauvreau , ScDa, Jane W. Newburger , MD, MPHa, James E. Lock , MDa, John P. Kupferschmid , MDb**, Janice Burnett a, Richard A. Jonas , MDb, Aldo R. Castañeda , MD, PhDb, Gil Wernovsky , MDa***

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

Received for publication Nov. ; revisions requested Feb. 13, 1997; revisions received April 14, 1997; accepted for publication April 16, 1997 Address for reprints: John E. Mayer, Jr., MD, Department of Cardiac Surgery, Children's Hospital, 300 Longwood Ave., Boston, MA 02115.

Abstract

Objective: The purpose of this study was to review a large, evolving, single-center experience with the Fontan operation and to determine risk factors influencing early and late outcome. Methods: The first 500 patients undergoing modifications of the Fontan operation at our institution were identified. Perioperative variables were recorded and a cross-sectional review of survivors was undertaken. Results: The incidence of early failure decreased from 27.1% in the first quartile of the experience to 7.5% in the last quartile. In a multivariate model, the following variables were associated with an increased probability of early failure: a mean preoperative pulmonary artery pressure of 19 mm Hg or more ( p < 0.001), younger age at operation ( p = 0.001), heterotaxy syndrome ( p = 0.03), a right-sided tricuspid valve as the only systemic atrioventricular valve ( p = 0.001), pulmonary artery distortion ( p = 0.04), an atriopulmonary connection originating at the right atrial body or appendage ( p = 0.001), the absence of a baffle fenestration ( p = 0.002), and longer cardiopulmonary bypass time ( p = 0.001). An increased probability of late failure was associated with the presence of a pacemaker before the Fontan operation ( p < 0.001). A morphologically left ventricle with normally related great arteries or a single right ventricle (excluding heterotaxy syndrome and hypoplastic left heart syndrome) were associated with a decreased probability of late failure ( p = 0.003). Conclusions: These analyses indicate that early failure has declined over the study period and that this decline is related in part to procedural modifications. A continuing late hazard phase is associated with few patient-related variables and does not appear related to procedural variables.




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