Raising Awareness About the Use of Validated Instruments to Assess Quality of Life in Patients With Pulmonary Arterial Hypertension

OBJECTIVES: To find out how often Health Related Quality of Life (HRQoL) is assessed in patients with Pulmonary Arterial Hypertension (PAH) at the time of diagnosis, as well as to elucidate which tools are most frequently used.

METHODS: Multicentric retrospective cross-sectional analysis of 3668 prevalent and incident PAH patients enrolled in the Spanish Registry of Pulmonary Arterial Hypertension (REHAP) from 2007 to 2023.

RESULTS: 3,668 prevalent and incident patients with PAH. Mostly female (67.8%), with a mean age of 50.2 years SD 18.1. PAH patients were distributed in Idiopathic (30.9%), Associated with Connective Tissue Disease (22.7%) and with Congenital Heart Disease (22.1%). Most of them were in NYHA functional class II and III (36.7% and 49.3%,respectively). The HRQoL of only 68 patients (1.9%) was analyzed, with the majority (63.2%) not specifying the tool used. Among those that were reported, generic tools were the most used (SF-36 in 16.2% and EQ5D in 8.8%). Only in 2 patients was reported a PAH-specific tool used, the EMPHASIS-10.

CONCLUSIONS: Among PAH patients included in REHAP, HRQoL questionnaires seem to be underused. Greater use of these instruments could increase interest in the implementation of non-pharmacological interventions and address the personal needs of a highly vulnerable population. For greater application in real life, specific, simple tools are needed, validated in Spanish and accessible to all professionals caring for patients with PAH.