ABSTRACT
BACKGROUND: Impaired health-related quality of life (HRQoL) is a common problem in pulmonary arterial hypertension (PAH), but there is limited data on HRQoL in children with PAH. We aimed to investigate the QoL, determine the potential risk factors for poor HRQoL in children with PAH, and assess the depression and anxiety of their families. METHODS: We performed a prospective cross-sectional study of children with PAH, healthy peers, and their parents. HRQoL was measured by the self-reported and age-adapted KINDL questionnaire. Beck Depression Inventory (BDI) and hospital anxiety and depression scale (HADS) were used to assess the depression and anxiety of parents. RESULTS: Children with PAH had statistically lower total HRQoL scores than healthy peers (p < 0.001). There was no correlation between HRQoL and duration of disease, World Health Organization functional class, pro-B-type natriuretic peptide, 6-min walk test, and combined or single treatment. BDI and HADS scores were significantly higher in the parents of patients (p < 0.001, p = 0.023, p < 0.001, respectively). There was a negative correlation between HRQoL and BDI in patients (p = 0.016), while there was no significant correlation between HRQoL and HADS (p > 0.05). CONCLUSION: We demonstrated impairment of HRQoL of children with PAH. In addition, there was a correlation between the depression of the families and the QoL of the children.
