Abstract 1869: Predictors of Morbidity and Mortality in Contemporary Fontan Patients: Results From a Multicenter Study Including Cardiopulmonary Exercise Testing in 321 Patients
Background: Previous studies have established an association between exercise intolerance and increased morbidity and mortality in patients with congenital heart disease.
Methods and Results: This retrospective study - including all Fontan patients who underwent cardiopulmonary exercise testing (CPET) at four major European Centers - aimed to clarify if reduced exercise capacity is associated with poor outcome in patients after Fontan palliation and to identify risk factors for mortality and cardiac-related hospitalization. In total, 321 patients (57% male, mean age 20.9±8.6 years) underwent CPET-testing between 1997 and 2008 and were included in the analysis. Median follow-up after CPET was 21 (IQR 13.8 to 41.5) months. During this period 22 patients died and 6 patients underwent cardiac transplantation. Parameters of CPET were strongly related to increased risk of hospitalization, but - with the exception of heart rate reserve - unrelated to risk of death. In contrast, patients with clinically relevant arrhythmia requiring antiarrhythmic medication had a 4.2 fold increased mortality risk (P=0.001). Furthermore, patients with atriopulmonary or atrioventricular Fontan had a 5.2 fold increased risk of death compared with TCPC patients (P=0.008). The combination of clinically relevant arrhythmia, “classic” Fontan and signs of symptomatic or decompensated heart failure requiring diuretic therapy was associated with a particularly poor outcome (5 year mortality 52 %).
Conclusions: Exercise intolerance is associated with increased morbidity but not with increased mortality in contemporary Fontan patients. Clinically relevant arrhythmia in the setting of a “classic” Fontan is associated with poor prognosis, potentially identifying patients requiring immediate medical and/or surgical attention.