Lung function and aerobic capacity in adult patients following modified Fontan procedure.

OBJECTIVE To examine cardiopulmonary performance in 52 adult patients with a Fontan circulation. DESIGN Retrospective cohort study. Values of maximum oxygen uptake (VO(2)max), maximum heart rate (HRmax), forced vital capacity (FVC), and forced expiratory volume in one second (FEV(1)) were compared with predictive values for different age groups. Patients were further subdivided into those with a pulmonary artery connection (RA-PA) or right atrium to right ventricle conduit (RA-RV). RESULTS At late follow up (median 10 years, range 1 to 26 years), patients with Fontan circulation had greatly diminished VO(2)max, HRmax, FVC, and FEV(1) compared with predicted values. Early age at surgery had a positive impact on aerobic capacity. The FEV(1):FVC ratio indicated restrictive lung function. No differences were found with respect to any variable between patients with RA-PA connections and those with RA-RV connections. CONCLUSIONS Patients with a Fontan circulation have greatly diminished values of aerobic capacity and a restrictive pattern of lung function. Patients with an early surgical procedure obtained higher values of VO(2)max. The theoretical benefits of including the right ventricle in a Fontan circulation were not apparent.