Background

Pulmonary Arterial Hypertension (PAH) is a rare and progressive disease. It can have a significant impact on patients? quality of live (QoL).

Objectives

Our aim was to investigate QoL in a cross-section of patients with PAH.

Methods

We analysed data from the Adelphi PAH Disease Specific Programme (DSP?); a real-world cross-sectional survey and chart review, to snapshot patient QoL across the US, Europe (France, Germany, Italy, Spain and the UK) and Japan. Data was collected between March and August 2022. QoL was measured with the EQ-5D-5L VAS (score 0-100, a higher score indicates better health) and the emPHasis-10 (10 items, the total max. score is 50, a lower score indicates better health). Scores are reported as means and standard deviations (SD) per functional class (FC) level (n=622).

Results

The EQ-5D was 77 for FC I to 43 for FC IV, indicating that a higher FC is associated with poorer QoL. The QoL score found for patients with FC I was lower than the value found for the general population in the US (84). The mean score for the emPHasis-10 score ranged from 15 to 39, while the difference in between scores between different FC levels was more than the reported minimally important difference of 6 points.

Conclusion

The QoL for patients with PAH was lower than the general population and decreased with a higher FC level. Improving patients? FC level has the potential to greatly improve patients QoL.

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