FUNCTIONAL OUTCOME AFTER THE FONTAN OPERATION - FACTORS INFLUENCING LATE MORBIDITY

Objectives: The purpose of this study was to describe the functional o utcome of a large number of patients after modifications of the Fontan operation and to investigate perioperative risk factors that might in fluence late functional state, Methods: A comprehensive cross-sectiona l review of the first 500 patients undergoing a Fontan operation at ou r institution was undertaken. Those surviving with an intact Fontan ci rculation were reviewed by questionnaire to assess functional status a nd medication history, Medical records, chest roentgenograms, echocard iograms, cardiac catheterizations, and laboratory investigations were also reviewed to assess postoperative status, Results: Three hundred s ixty-three long-term survivors with an intact Fontan circulation were identified during cross-sectional follow-up, Median age at operation w as 5.0 years (range 0.4 to 31 gears), and median follow-up was 5.4 yea rs (range 1.7 to 20 years), Most patients (91.1%) were in New York Hea rt 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 aerial septectomy (p = 0.03), and a prior mai n pulmonary artery-ascending aorta anastomosis (p = 0.05), Conclusions : A poor functional outcome is uncommon after the Fontan operation bur becomes more frequent with increasing duration of follow-up.