Real-world experience with selexipag in patients with pulmonary arterial hypertension in North America and Europe

Real-world data contribute to understanding the treatment landscape of rare diseases such as pulmonary arterial hypertension (PAH). SPHERE (<ext-link>NCT03278002</ext-link>; US; completed) and EXPOSURE (EUPAS19085; Europe and Canada; ongoing, data cut-off Nov 2021) are two large, prospective, multicentre observational studies on PAH. The aim of this pooled analysis was to describe real-world experience with selexipag, a selective oral prostacyclin receptor agonist. Overall, 894 patients had newly initiated selexipag. Median (Q1, Q3) age was 61 (48, 70) years and time since diagnosis was 2.7 (0.8, 7.4) years. Most patients were female (74%) with idiopathic PAH (54%) and initiated selexipag as triple oral therapy (67%). Median (Q1, Q3) selexipag exposure was 10.8 (3.3, 18.6) months. Following titration, 41% (307/742) of patients discontinued selexipag, with 150 (20%) discontinuations due to tolerability and 46 (6%) due to death. Overall, 8% (70/894) of patients died. A total of 591 patients had 1-year mortality risk score (2022 ESC/ERS 4-strata risk assessment) available at selexipag initiation. The 1-year Kaplan-Meier (KM) survival estimates were 100% for low-risk patients, 96% for intermediate-low, 91% for intermediate-high, and 59% for high-risk (Figure 1). In North American and European clinical practices, patients initiated selexipag across all risk strata, with an overall 1-year KM survival estimate of 93%. <fig><object-id>erj;64/suppl_68/PA4319/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>