LAUSR

Abstract Objective Fontan survivors report reduced health-related quality of life (HRQOL) and have decreased exercise tolerance compared to healthy peers. We recently demonstrated that a 12-week home-based physical activity program was safe, feasible, and improved parent proxy-reported HRQOL and objective measures of exercise capacity in patients with Fontan circulation. We sought to determine if these improvements in HRQOL and exercise capacity are sustained at 6-month follow-up. Design Patients, 8–12 years old, with Fontan circulation that completed a 12-week moderate-to-vigorous intensity home-based physical activity program were invited to attend a 6-month follow-up session to complete objective assessments of HRQOL and exercise capacity. HRQOL was measured with validated questionnaires. The 20-meter Shuttle Run (PACER Test) was used to measure exercise capacity. Results Of the 13 patients who completed the original 12-week physical activity program, 11 (85%) attended the 6-month follow-up. There were no adverse events during the follow-up period. Improvements in parent proxy-report for HRQOL at completion of the 12-week program were sustained at 6-month follow-up. Patients reported a significant decrease in HRQOL assessed by PedsQL at 6-month follow-up. However, there was a non-significant trend of improvement in patient PCQLI total and psychosocial impact scores at completion of the 12-week program, which was sustained at 6-month follow-up. The significant improvements in objective measures of exercise capacity seen from baseline to completion of the 12-week program were sustained at 6-month follow-up. Conclusion Improvements in parent-proxy report HRQOL and objective measures of exercise capacity after completion of a home-based 12-week physical activity program were sustained at 6-month follow-up. Despite improvements in exercise capacity, patients did not report improved HRQOL. A larger, controlled study of this home-based physical activity program is needed to assess the impact of physical activity and improved exercise capacity on HRQOL in children with Fontan circulation.