Quality of life of patients with pulmonary arterial hypertension: a meta-analysis

Abstract

Objective: Pulmonary arterial hypertension (PAH) is a rare condition, with an incidence of 15-50 cases per million annually. Available studies demonstrate that despite the longer survival of PAH patients, their quality of life (QoL) deteriorates as the condition progresses. Consequently, the goals of PAH therapy have expanded from increasing survival to improving health-related quality of life. The objective of this systematic review and meta-analysis was to summarize the available evidence about the level of QoL in patients with PAH.

Materials and methods: A systematic search was performed using the Cochrane guidelines for conducting meta-analysis following the PRISMA statement. The meta-analysis includes findings from 11 studies evaluating the QoL of PAH patients at baseline and at follow-up (12 weeks) using the Short Form (36) Health Survey (SF-36), the Minnesota Living with Heart Failure Questionnaire (MLHFQ) and the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR).

Results: The mean physical component score (SF-36) for the group was 37.2 points (95% CI: 33.24-41.16) and the heterogeneity coefficient was I²=97.71% (p < 0.001). The mean mental component score (SF-36) was 46.38 (95% CI: 44.21-48.56) and the heterogeneity coefficient was I²=87.92% (p < 0.001). The result indicates improved QoL 12 weeks after the intervention, though three papers did not fully confirm this. The greatest improvement in QoL was found in patients treated with bosentan and iloprost and the smallest improvement in QoL was found in patients treated with epoprostenol sodium. The heterogeneity coefficient was I²=91.36%, p < 0.001 for CAMPHOR and I²=97.65%, p < 0.001 for MLHFQ.

Conclusions: PAH patients tend to have a poor QoL, mainly in the physical functioning domain, less so in the psychological functioning domain. QoL may be improved by therapeutic interventions, mainly pharmaceutical ones. Patients with PAH also tend to suffer from depression, anxiety, stress, or sleep disorders. All these factors are significantly correlated with poorer QoL.