Rationale: The development of evidence-based treatment guidelines for pediatric pulmonary arterial hypertension (PAH) is hampered by lack of pediatric clinical trials. Trial design is hampered by lack of a feasible clinical endpoint in this population.
Objectives: To evaluate the use of accelerometry for measuring physical activity (PA) in pediatric PAH and to investigate its correlation with clinical disease severity markers.
Methods: We included children from the Dutch National Network for Pediatric Pulmonary Hypertension. Control patients were recruited from the outpatient cardiology clinic of the Beatrix Children's Hospital. Children were asked to wear the accelerometer for 7 days. Vector magnitude counts per minute (VM CPM) and time per day spent in different PA intensity levels were defined as accelerometer outcomes.
Measurements and Main Results: VM CPM was lower in children with PAH (n = 29) than in controls (n = 60; 647 vs. 921; P < 0.001). Children with PAH spent less time in moderate and vigorous PA (13 vs. 29 min/d and 2 vs. 13 min/d, respectively; P < 0.001). Time spent in moderate and vigorous PA correlated inversely with World Health Organization functional class. Time spent in moderate PA correlated positively with 6-minute-walk distance. In post hoc analyses, VM CPM and time spent in moderate/vigorous combined and vigorous PA were associated with outcome (P ≤ 0.044).
Conclusions: PA is markedly decreased in children with PAH. Accelerometer output correlated with clinical disease severity markers and may predict outcome. We showed an exciting potential of PA as a meaningful endpoint for clinical trials in pediatric PAH, although its clinical utility and prognostic value need to be further validated.