Positive pediatric exercise capacity trajectory predicts better adult Fontan physiology rationale for early establishment of exercise habits

Abstract Objective Exercise training is recommended for its possible favorable effects on Fontan pathophysiology. This study aimed to elucidate the impact of pediatric exercise capacity trajectory, which may mimic the effect of exercise training, on late adult Fontan pathophysiology. Methods Since 1990, 97 Fontan patients had consecutively undergone two serial cardiopulmonary exercise tests (CPX1 and CPX2) during childhood (ages 8 ± 2 and 14 ± 2 years) and one during adulthood (CPX3; age 23 ± 5 years). The changes in peak oxygen uptake (PVO 2 : % of normal value) from CPX1 to CPX2 (1-dPVO 2 ) and from CPX2 to CPX3 (2-dPVO 2 ) were calculated, and then the patients were divided into four subgroups according the 1-dPVO 2 and 2-dPVO 2 . Results In their adulthood, when compared with groups with negative 1-dPVO 2 , the central venous pressure, plasma brain natriuretic peptide level, and renal resistive index were lower, whereas liver synthetic function, body fat-free percentage, and PVO 2 were higher in those with positive 1-dPVO 2 ( p 2 with adult Fontan pathophysiology were not observed, except for the PVO 2 . After CPX3, 13 unexpected events occurred, and the risk was 76% lower in the groups having positive 1-dPVO 2 than in those with negative 1-dPVO 2 (hazard ratio, 0.24; 95% confidence interval, 0.09–0.62; p  = 0.0035). Conclusions A positive exercise capacity trajectory during childhood predicts better adult Fontan pathophysiology, including better prognosis. Thus, prescription of exercise could be a promising strategy in the management of pediatric Fontan patients.