Children with pulmonary hypertension (PH) engage in less physical activity than their peers. This is a concern since adult data support exercise as a non-pharmacologic treatment for PH. Despite adult data, therapeutic exercise has not been widely adopted in pediatric PH. Investigators have previously demonstrated that children with PH have less skeletal muscle mass in association with worse exercise performance. Interventions to increase physical activity and skeletal muscle mass may improve exercise performance and quality of life in children with PH. This study will use wearable activity monitoring devices to promote physical activity in a 16-week pilot intervention in children and teenager with PH.
Age 8-18 years
PH World Health Organization (WHO) diagnostic groups 1, 2, 3, or 4 (pulmonary arterial hypertension, PH due to left heart disease, PH due to lung disease, chronic thromboembolic PH)
WHO functional class I or II
Ambulatory status
WHO functional class III or IV
Single ventricle physiology
Moderate to severe renal disease (>stage 3)
Severe hepatic impairment [aspartate aminotransferase (AST)/alanine transaminase (ALT) > 2x upper limit of normal]
Single group
Exercise intervention
Behavioral16-week home exercise intervention, enriched by mobile health technology
Change in moderate to vigorous physical activity (MVPA)
Change in MVPA (min/day) will be measured by smartwatch accelerometer from baseline over the course of the intervention
Change in leg lean mass Z-score (LLMZ)
LLMZ will be measured by densitometry. LLMZ is a percentile score with scores -2 to 2 considered typical
Change in muscle strength
Upper extremity strength will be measured by handgrip dynamometer. Lower extremity strength will be measured by Biodex across the knee and ankle
Change in quality of life score
Score measured by completion of PedsQL questionnaire. Scores range from 0-100 with higher scores indicating better quality of life
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Children's Hospital of Philadelphia
Lead sponsor
National Heart, Lung, and Blood Institute (NHLBI)
Collaborator
