Despite improvement in survival, patients with a Fontan circulation are at risk for numerous complications, and exercise capacity and quality of life are usually reduced compared with healthy controls. However, only a few studies have assessed the impact of the amount of sporting activity on exercise capacity and health-related quality of life. We analyzed cardiopulmonary exercise tests (CPET) in a large cohort of patients with a Fontan circulation. Questionnaires were used to assess health-related quality of life and sporting activity. A total of 79 patients with a median age of 13.0 (6.5 to 34.4) years at CPET were included (female, n = 31). Questionnaires revealed that 80% of patients (n = 63) do leisure sports with 43% (n = 27) exercising more than 2 hours per /week. In a subgroup analysis on pediatric patients (n = 52) we found that nearly all participate in school sports (n = 51) and report good subjective health (n = 48). In the pediatric subgroup, oxygen uptake at the anaerobic threshold and peak oxygen uptake correlated with subjective health (p <0.05) and the amount of leisure sports activity (p <0.01). In the overall cohort, running time and running distance were significantly associated with the hours of sports per week (p <0.01). CPET results did not significantly differ between single right and single left ventricle patients. Furthermore, an open fenestration was not associated with reduced exercise capacity. In conclusion, most patients with a Fontan circulation participate in leisure sports and report good subjective healthiness. In pediatric patients, increased sports activity is associated with better exercise capacity and subjective healthiness.
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