Quality of life in patients with pulmonary arterial hypertension: A systematic review and meta-analysis

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare incurable disease characterized by pulmonary artery remodeling, which may eventually lead to right ventricular failure and death. Despite advancements in targeted therapies, PAH remains life-threatening with significant impact on patients’ quality of life (QoL). The importance of patient-reported outcome measures (PROMs) in assessing health-related QoL has been increasingly recognized, but their implementation remains inconsistent in clinical practice. This systematic review aims to assess and synthesize the available evidence on the impact of PAH on patients’ QoL measured by PROMs. METHODS: A systematic review was conducted following PRISMA guidelines. Studies were identified through searches in CINAHL, EMBASE, Web of Science, PubMed, Scopus, and BVS. Inclusion criteria encompassed observational studies and RCTs with non-pharmacological interventions assessing QoL in PAH patients. RCTs involving drugs were excluded. Risk of bias was assessed using the Joanna Briggs Institute (JBI) appraisal tools. A meta-analysis was performed using a random-effects model. RESULTS: A total of 3271 studies were retrieved; 44 met inclusion criteria. PROMs like SF-36 (14 studies), SF-12 (2), CAMPHOR (9), EQ-5D (7), and EmPHasis-10 (13) were utilized. Findings highlight a dichotomy in QoL outcomes, with significant physical impairments but relatively preserved mental health scores. Subgroup analyses revealed substantial heterogeneity, emphasizing the need for standardized tools. CONCLUSIONS: This review underscores the critical role of PROMs in understanding QoL in PAH patients. Findings advocate for integrating PROMs into clinical assessments and standardizing methodologies. REGISTRATION: The protocol was registered in PROSPERO (CRD42022292145).