PAH remains a progressive, life-limiting disease driven by elevated pulmonary vascular resistance and right-heart strain. A single-center, retrospective study from Shandong University Qilu Hospital evaluated outcomes with selexipag-based triple targeted therapy in adults with Group 1 PAH confirmed by right-heart catheterization.
From February 2022 to November 2023, 228 consecutive patients were screened and 146 were included in the effectiveness cohort. The primary endpoint was improvement in the number of low-risk indices, defined as World Health Organization functional class I/II, six-minute walk distance greater than 440 meters, and NT-proBNP less than 300 pg/mL, assessed 3 to 6 months after selexipag initiation.
The proportion of patients meeting all three indices increased from 12.3% at baseline to 50.7% at follow-up. Patients starting initial triple therapy demonstrated significantly better hemodynamic response and right-heart remodeling than those receiving sequential add-on treatment, with greater reductions in right atrial area (−5 vs −1 cm²; p = 0.013), right ventricular diameter (−8 vs −3 mm; p = 0.001), and improved tricuspid annular plane systolic excursion (+3 vs +1 mm; p = 0.002). Selexipag-based therapy was well tolerated throughout follow-up.
These findings highlight the clinical value of early triple combination therapy for enhancing functional capacity, right-heart function, and overall risk profile in patients with PAH. Larger prospective studies are needed to confirm durability and long-term outcomes.