Abstract
BACKGROUND AND OBJECTIVES: As long-term survival after the Fontan operation has improved, exercise capacity has become a crucial determinant of prognosis. Various exercise rehabilitation programs involving different populations and protocols have been developed to improve these outcomes. This systematic review and meta-analysis compared the effects of exercise rehabilitation in patients with Fontan circulation according to age group and exercise method. METHODS: We searched the PubMed, Embase, and Cochrane Library databases for articles on exercise rehabilitation programs for patients who had undergone the Fontan procedure up to November 2023. After selection and eligibility assessment, 20 studies (5 randomized controlled trials [RCTs], 2 randomized trials, and 13 cohort studies) were included in the meta-analysis of peak oxygen consumption (VO₂) and minute ventilation equivalents for carbon dioxide production (VE/VCO₂ slope). RESULTS: Peak VO₂ was significantly better in groups with exercise training than in the control groups in 5 RCTs (standardized mean difference [SMD], 0.48; p=0.0017); it showed a notable increase in 20 studies before and after exercise training (SMD, 0.44; p=0.001). VE/VCO₂ showed no significant changes in the RCTs (SMD, 0.22; p=0.68) or before and after exercise training (SMD, -0.11; p=0.25). Subgroup analyses revealed significant improvements in peak VO₂ for aerobic exercise (SMD, 0.32; p=0.0136) and in groups that included children (SMD, 0.49; p=0.0013 in "children and adults" and SMD, 0.49; p=0.047 in "children" group). CONCLUSIONS: Exercise training is effective for improving exercise capacity in patients after the Fontan procedure, particularly when initiated at a young age and implemented as an aerobic exercise.