Appraisal and Evaluation of the quality of life in Pulmonary Arterial Hypertension Instruments: A Systematic Review Using COSMIN Methodology.

ABSTRACT

AIM: The aim of this review was to identify specific instruments currently available for measuring quality of life in patients with pulmonary arterial hypertension (PAH) and to evaluate their psychometric properties in order to provide robust evidence for their application in clinical practice. BACKGROUND: Pulmonary arterial hypertension is a rare pulmonary vascular disorder predominantly affecting women aged 30-50 years. It leads to elevated pulmonary artery pressure, causing increased cardiac workload. Symptoms such as dyspnea and fatigue progressively deteriorate. Given the substantial impact on patient well-being, quality of life assessment is a critical concern. Generic quality of life measures often fail to capture the unique challenges associated with PAH. Therefore, identifying a PAH-specific quality of life instrument is essential for optimising patient management. DESIGN: A systematic literature review. METHODS: A systematic review was performed to assess the psychometric properties of quality of life instruments for PAH patients, following the 2018 Consensus-based Standards for the Selection of Health Measurement Instruments (COSMIN) guidelines. The literature search was conducted across Pubmed, Scopus, CINAHL, EMBASE, and APA PsycINFO databases. RESULTS: This review included four quality of life instruments CAMPHOR, LPHQ, emPHasis-10, and PAH-SYMPACT. CAMPHOR and PAH-SYMPACT received a GRADE A rating, while LPHQ and emPHasis-10 were rated GRADE B. Despite some sample size limitations, these instruments demonstrated varying degrees of internal reliability, validity, and content coverage for assessing quality of life in pulmonary arterial hypertension patients. CONCLUSIONS: This review provides an overview of available tools for assessing quality of life in patients with pulmonary arterial hypertension. Critical evaluation of these tools highlights incomplete psychometric assessments and methodological limitations in reference studies. Future research should prioritise more rigorous methodologies to ensure comprehensive psychometric evaluations. REGISTRATION NUMBER At point of submission put this on title page TWEETABLE ABSTRACT an optional additional 140 character message that can be 'tweeted'.