Abstract 2602: Results of Atrioventricular Valve Surgery following Modified Fontan Operation. The Mayo Clinic 36 Year Experience
Atrioventricular (AV) valve surgery after Fontan operation is a marker of poor outcome. Systemic AV valve issues are dealt with prior to Fontan operation. But, there are scant long-term data for patients that had AV valve surgery after Fontan. We evaluated early and late results of AV valve surgery after Fontan operation. Variables traditionally known to correlate with poor outcome, and current health status of survivors were assessed. All patients who underwent AV valve surgery after Fontan operation were identified from the Mayo Clinic Fontan database (1972–2008). Records were abstracted for preoperative, operative, and postoperative data. All patients not known to be deceased were sent health status questionnaires. A total of 61 patients (28 females) with a modified Fontan operation had subsequent AV valve surgery. Median age at operation was 14 years (2 – 41 years). The AV valve was replaced in 23 (38%) and the remaining had repair. Median duration between Fontan and AV valve surgery was 4.7 years (<1– 20.2 years). Median follow-up was 9 years (0 – 28 years). Heterotaxia was present in 11 (18%) patients. There were a total of 32 deaths (53%), 8 (25%) occurred within 30 days of operation or before hospital discharge. Twelve (38%) patients died of heart failure, and 4(13%) underwent cardiac transplant. Concomitant surgeries were performed in 37 (62%) patients. The 5-, 10-, and 15-year survival was 67%, 49%, and 36%, respectively. Median survival following surgery was 14.5 years. Thirty four percent reported having symptoms (NYHA class = 2– 4). However, 44 (72%) had arrhythmias, 9 (14.7%) had a thromboembolic event, and 12 (19.6%) developed protein-losing enteropathy (PLE). Ventricular dysfunction before surgery, left ventricle and mitral valve morphology and PLE were risk factors associated with death. Late morbidity and mortality following AV valve surgery after Fontan procedure are substantial. The incidence of PLE and arrhythmias in this cohort is substantial. This study, for the first time, demonstrates the poor long-term outcome of patients having AV valve surgery after the Fontan operation. If possible, issues related to the systemic AV valve should be addressed prior to Fontan procedure.