Assessment of quality of life in pediatric patients with pulmonary hypertension

Abstract

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 2–18 years with PH. Parents of all children and patients aged 8–18 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 2–4 years; three were aged 5–7 years; 11 were aged 8–12 years, and 12 were aged 13–18 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.

Health‐related quality of life and parental depression in children with pulmonary arterial hypertension

“…[22][23][24] We used KINDL since there was no specific scale for children with PAH. Similar to other children's study, 12,13 our study demonstrated that children with PAH had statistically lower total HRQoL scores than healthy peers. In addition, subscales scores of physical well-being, emotional well-being, selfesteem, family, friends, and everyday functioning were lower.…”

“…Many studies demonstrated that the QoL is impaired in adults with PAH, while studies in children with PAH are limited. 12,13 Our patient group was more homogeneous compared to other studies. 74% of the patients were PAH-CHD, and 26% were IPAH.…”

“…8,9 While there are many adult studies on QoL in PAH, 10,11 limited data exist regarding QoL in children with PAH. 12,13 The current study mainly aimed to investigate the QoL and determine the potential risk factors for poor HRQoL in children with PAH. Second, we aimed to assess the relationship between families' depression and anxiety and children's QoL.…”

Bronchopulmonary dysplasia

“…Although numerous studies have addressed the short-term and long-term effects of BPD on respiratory QOL, few if any have accounted for the additive effects of PVD. Although not specific to BPD, QOL surveys of parents of children with pulmonary hypertension who were treated with pulmonary vasodilators have found substantially lower QOL scores and higher parental stress, with scores even worse than those reported in children with cancer or congenital heart disease 244,245 . Limited data indicate that treatment of BPD-related pulmonary hypertension correlates with improvements in functional status and thus presumably QOL 246 .…”

Off‐label use of PAH‐targeted medications approved for adults and their financial coverage by health insurances are vital for children with pulmonary hypertension

“…Quality of life for paediatric patients with pulmonary hypertension is rated lower by parents and patients than quality of life associated with congenital heart disease or paediatric cancer. 62 Especially in the young, clinical deteriorations in the context of pulmonary hypertensive vascular disease and associated RV dysfunction usually lead to an increased number of outpatient contacts, and to costly, hospital admissions, frequently requiring intensive care monitoring and therapy. Thus, even from an economic standpoint, health insurance coverage of off-label combinatory pharmacotherapy for paediatric PH is likely a worthwhile financial commitment.…”