The Journal of pediatrics,2026 Oct 23
- Objective: To examine how measures of cardiac, vascular, and muscle health relate to Pediatric Quality of Life Inventory TM (PedsQL) score in a cohort of children and adolescents with Fontan circulation.
- Study Design: We performed a cross-sectional analysis of baseline data from children and adolescents (8-19 years) with Fontan circulation enrolled in a prospective study. Participants underwent a series of tests, including a cardiopulmonary exercise test, dual-energy X-ray absorptiometry measures of appendicular lean mass index (ALMI, kg/m2 ), leg dynamometry, and the 23-item PedsQL. The survey quantifies Physical, Emotional, Social, and School functioning into 1 total score and 2 summary scores for physical and psychosocial health.
- Results: 135 patients (median age 12.7 [IQR 10.2, 15.6] years) completed the baseline visit. Median time from Fontan operation was 8.7 years [IQR 5.9, 12.0]. Peak VO2 was 1309 [IQR 1070, 1670] mL/min, and indexed peak VO2 was 31.1 [IQR 25.2, 36.1] mL/kg/min. Physical and Psychosocial PedsQL summary scores were 78.1 [IQR 67.2,90.6] and 76.7 [IQR 63.3, 90], respectively, and the total score was 76.1 [IQR 65.2, 88.0]. Absolute peak VO2 was weakly correlated with PedsQL Physical Summary Score (ρ=0.21, P=0.015), but indexed peak VO2, % predicted peak VO2, and Fontan-specific peak VO2 z-score were not ALMI z-score was -0.84 [IQR -1.56, -0.14] and positively correlated with total PedsQL score (ρ=0.28, P=0.001) and the Physical Functioning domain (ρ=0.30, P<0.001).
- Conclusions: Skeletal muscle mass was associated with quality of life in children with Fontan circulation. The association between VO2 and physical function was attenuated with body-size normalization.