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A recent systematic review assessed the psychometric properties of various instruments for measuring QOL in patients with pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) has a substantial impact on patient well-being. However, there is no gold standard for QOL assessments in patients with this rare pulmonary vascular disorder, and no systematic reviews have rigorously assessed QOL instruments in PAH.

In new research published in Respiratory Medicine, Irene Gangemi and colleagues performed a systematic literature review assessing the psychometric properties of QOL instruments for patients with PAH, following the 2018 Consensus-based Standards for the Selection of Health Measurement Instruments (COSMIN) guidelines. The eligibility criteria included studies on developing and validating QOL measurement instruments in patients with PAH; articles published in English, Italian, and Spanish; and peer-reviewed articles published in academic journals.

The review included 20 studies and four QOL instruments: Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR), PAH-SYMPACT, Living with Pulmonary Hypertension Questionnaire (LPHQ), and emPHasis-10. The quality of the evidence (high, moderate, low, and very low) for each instrument was rated using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE).

According to the COSMIN methodology, CAMPHOR and PAH-SYMPACT demonstrated better psychometric properties and received a GRADE A rating. These instruments can capture and evaluate specific domains, such as symptoms and their impact on the QOL of these patients. LPHQ and emPHasis-10 were rated GRADE B.

The authors identified several key areas regarding the impact on QOL. For the social well-being domain, only the CAMPHOR and LPHQ questionnaires address the difficulty of sustaining relationships with family and friends. For physical well-being, most instruments assess basic limitations. However, CAMPHOR measures the need to plan activities and the perception of losing control over one’s body. LPHQ also evaluates additional aspects, such as sleep issues.

PAH-SYMPACT and LPHQ are the only tools that evaluate mental well-being, and CAMPHOR and LPHQ are the only instruments investigating financial well-being.

“The clinical use of the included scales requires critical evaluation, in light of an incomplete psychometric evaluation and, sometimes, questionable or inadequate methodology in reference studies,” concluded Green and colleagues.

“The difference in the validation levels of the psychometric tools mainly depends on the rigor and completeness of the methodology used to evaluate the tool itself. It is necessary that future research focuses on the development of scales using a more rigorous methodology employing higher quality methods and estimating all psychometric properties.”