%0 Journal Article %T Assessment of quality of life in pediatric patients with pulmonary hypertension %A Brian J. Hallis %A Cheryl L. Brosig %A Edward C. Kirkpatrick %A Evelyn M. Kuhn %A Kathryn A. Tillman %A Mary Krolikowski %A Stephanie S. Handler %J Pulmonary Circulation %@ 2045-8940 %D 2019 %R 10.1177/2045894018822985 %X The aim of this study is to evaluate quality of life in four domains (physical, emotional, social, and school) in pediatric patients with pulmonary hypertension (PH) using a validated survey (PedsQL). This is a prospective cohort study of pediatric patients aged 2每18 years with PH. Parents of all children and patients aged 8每18 years with appropriate developmental capacity completed the PedsQL survey in the clinic. Results were compared with published norms for pediatric patients, those with congenital heart disease (CHD) and cancer. Thirty-three children were enrolled yielding 32 parent and 18 patient self-reports: seven patients were aged 2每4 years; three were aged 5每7 years; 11 were aged 8每12 years, and 12 were aged 13每18 years. Twenty-one patients were classified as World Health Organization (WHO) Group I pulmonary arterial hypertension (PAH), 11 WHO Group III PH due to lung disease, and one WHO Group V with segmental PH. Thirteen patients were NYHA functional class (FC) 1, 12 were FC 2, eight were FC 3, and none were FC 4. The PH cohort had significantly lower scores than healthy children in all domains on both parent and self-report. The PH cohort also had significantly lower scores than patients with CHD (parent report: total, physical, social, school; patient self-report: total, physical, school) and cancer (parent report: school; patient self-report: physical, school). Close to 50% of participants reported at risk scores in each domain. The quality of life in pediatric PH patients assessed by PedsQL revealed functional impairment in multiple domains. Administration of the PedsQL during outpatient encounters may provide an easy, reproducible method to assess quality of life and direct referral for interventional services %K functional ability/impairment/quality of life/physical activity %K pediatric cardiovascular disease %K pulmonary hypertension %U https://journals.sagepub.com/doi/full/10.1177/2045894018822985