Abstract
Pulmonary hypertension (PH) in children requires complex medical management. Health-related quality of life (HRQoL) remains understudied in this population. During an 8-month period children and parents attending PH outpatient appointments completed the generic PedsQL (measuring physical, emotional, social, and school functioning). Parents completed the Hospital Anxiety and Depression scale, a validated measure of anxiety and depression, about their own mental health. Clinical data were extracted from the medical notes. Analyses explored relationships between clinical factors, parental mental health and HRQoL and compared scores with published norms. Parents of 94 of 98 (96%) eligible children with PH and 48 of 54 (89%) eligible children aged ≥ 5 years completed the PedsQL. All HRQoL scores were significantly below healthy norms, with 49% scoring > 2 S.D. below normative means. Physical HRQoL was associated with disease severity and survival outcomes. Multiple regression analyses showed age, learning disability, functional class, and parental depression explained 38% of parent-reported HRQoL variance (F(6, 86) = 7.67; p < 0.001) while learning disability explained 33% of child-reported variance (F(3, 45) = 6.78; p < 0.001). These findings support routine HRQoL evaluation and development of disease-specific measures for paediatric PH.