OBJECTIVES: The aim of the present study is to determine the long-term effects of a ten-week exercise training program in adult patients with a systemic right ventricle. METHODS: All patients who participated in a 2009 randomized controlled trial were approached. At approximately three years of follow-up from initial baseline, patients underwent cardiopulmonary exercise testing, filled out two quality of life questionnaires, and NT proBNP levels were measured. All examinations were performed according to the protocols of the 2009 trial. In addition, patients were asked about their current sports habits. RESULTS: Of the 54 patients who were randomized in the 2009-trial 40 participated in the current re-evaluation (male 50%, ccTGA 35%, age 36 +/- 10years, intervention group n = 22, control group n = 18). After three years, no persistent effect of exercise training on VO2peak training remained (-2% of predicted, 95% CI -3% to 5%; p = 0.56). However, patients who already participated in regular sports or exercise at baseline (n = 23/40 (58%)) showed higher VO2peak of 13% of predicted (95% CI 4% to 23%; p > 0.01) and a decrease of 62% in plasma NT-proBNP (95% CI -115% to -10%; p > 0.03) during follow-up, when compared to patients who did not. Moreover, sports were associated with a lower incidence of clinical events (p = 0.032). CONCLUSION: Short-term beneficial effects of exercise training did not persist over a three-year follow-up period. However, sports participation at baseline was associated with better exercise capacity, lower neurohormone levels, and increased event-free survival.

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