MODIFIED FONTAN OPERATION IN THE PRESENCE OF ANOMALIES OF SYSTEMIC AND PULMONARY VENOUS CONNECTION

Background. The purpose of this report is to outline technical maneuve rs dictated by anomalies of systemic and/or pulmonary venous connectio n in the performance of the Fontan procedure. Methods and Results. Bet ween 1975 and 1990, 104 patients (60 male, 44 female) with anomalies o f systemic and/or pulmonary venous connection underwent a modified Fon tan procedure at the Mayo Clinic. Mean age was 9.7+/-5.7 years. Isolat ed anomalies of the systemic venous connection were identified in 46 p atients, isolated anomalous pulmonary venous connections in 4, and a c ombination of the two in 54. Previous palliative operations had been p erformed in 93 patients. Surgical repair was accomplished by atrial se ptation or placement of an intra-atrial conduit combined with cavopulm onary anastomosis if required. Survival by Kaplan-Meier, including ope rative mortality, was 55.7% at 10.3 years, not significantly different from the overall survival of the Fontan population. By the proportion al hazards general linear model procedure, insufficiency of the system ic atrioventricular valve, preoperative mean pulmonary pressure greate r than 15 mm Hg, and pulmonary artery resistance index greater than 4 U . m2 were associated with higher mortality. Five patients required r eoperation for pulmonary venous obstruction (I patient), revision of t he atrial baffle (1 patient), revision of the intra-atrial conduit (2 patients), and replacement of the systemic atrioventricular valve (1 p atient). Conclusions. We conclude that the modified Fontan operation c an be successfully performed in this subset of patients, with long-ter m results comparable to those obtained in patients with normal systemi c and pulmonary venous connection.