Real-World Experience with Selexipag in Patients with Pulmonary Arterial Hypertension: Treatment Patterns and...

Treatment strategies for pulmonary arterial hypertension (PAH) depend on patients' 1-year mortality risk; however, real-world practices and outcomes remain inadequately described.

We aimed to investigate real-world experience with selexipag, an oral selective prostacyclin I2 receptor agonist used to treat PAH.

This analysis of two large, prospective, observational studies: SPHERE (NCT03278002; USA; completed) and EXPOSURE (EUPAS19085; Europe and Canada; ongoing, data cutoff November 2021) included adults newly initiating selexipag with follow-up available.

Of 1366 selexipag users, 894 met eligibility criteria. At selexipag initiation, the median (Q1-Q3) age was 61 (48-70) years and the time since diagnosis was 2.7 (0.8-7.4) years. Most patients initiated selexipag within triple oral combination therapy (67%) with WHO-FC III symptoms (61%). During a median 10.8 (Q1-Q3: 3.3-18.6) months of selexipag exposure, 388 (43%) patients discontinued selexipag, mostly as a result of tolerability (n = 173, 19%) and death (n = 69, 8%). The median individualised dose was 800 μg twice-daily (n = 742). One-year mortality risk at selexipag initiation (assessable for 591 patients using a four-strata method): 22% were low-risk, 40% intermediate-low, 29% intermediate-high, and 9% high-risk. From low to high risk: 86%, 73%, 57% and 45% were free from all-cause hospitalisation at 1 year, and 1-year survival was 100%, 96%, 91%, and 59%, respectively.

Despite current guidelines, over half of patients started selexipag almost 3 years after diagnosis, and 38% were already intermediate-high or high risk of 1-year mortality. Lower hospitalisation rates and better survival were observed for selexipag-treated patients in the low and intermediate-low risk groups versus higher risk groups.