Contemporary Outcomes After the Fontan Procedure

Abstract

Background The characteristics of contemporary Fontan survivors are not well described. Objective We characterized a large cohort of children who had a Fontan procedure, using measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP). Methods We enrolled 546 children (6–18 years, mean 11.9 years) and compared them within pre-specified anatomic and procedure subgroups. History and outcome measures were obtained within a three month period. Results Predominant ventricular morphology was left (LV) 49%, right (RV) 34%, and mixed 19%. Ejection fraction (EF) was normal for 73% of subjects; diastolic function grade was normal for 28%. Child Health Questionnaire mean summary scores were lower than for controls; however, over 80% of subjects were in the normal range. BNP concentration ranged from <4–652 pg/mL (median 13). Mean percent predicted peak oxygen consumption was 65% and decreased with age. EF and EF z-score were lowest, and semilunar and atrioventricular (AV) valve regurgitation were more prevalent in the RV subgroup. Older age at Fontan was associated with more severe AV valve regurgitation. Most outcomes were not associated with a superior cavopulmonary connection prior to Fontan. Conclusions Measures of ventricular systolic function and functional health status, although lower on average in the cohort compared to controls, were in the majority of subjects within two standard deviations of the mean for controls. RV morphology was associated with poorer ventricular and valvar function. Effective strategies to preserve ventricular and valvar function, particularly for patients with RV morphology, are needed.