Long-Term Outcomes of Selexipag in Schistosomiasis-Associated Pulmonary Arterial Hypertension

Overview

Schistosomiasis-associated pulmonary arterial hypertension is a serious condition that can lead to shortness of breath, heart failure, frequent hospitalizations, and early death. Although treatments for pulmonary arterial hypertension have improved over time, patients with this specific cause of the disease are often not included in long-term studies. Selexipag is an oral medication used to treat pulmonary arterial hypertension and is part of routine clinical care in Brazil. Its long-term effects in patients with schistosomiasis-associated pulmonary arterial hypertension are not well understood. The PROPULSE-Sch study aims to evaluate long-term clinical outcomes in patients with schistosomiasis-associated pulmonary arterial hypertension who received selexipag, compared with similar patients who did not receive this medication before it became available at the study center. This is an observational study using data from routine medical care. All treatments are prescribed by the treating physicians, and participation in the study does not change patient care. The results may help improve understanding of long-term outcomes and support treatment decisions in this population.

Schistosomiasis-associated pulmonary arterial hypertension (PAH-Sch) is a prevalent cause of pulmonary arterial hypertension in endemic regions and is associated with significant morbidity and premature mortality. Despite advances in targeted therapies for pulmonary arterial hypertension, patients with PAH-Sch remain underrepresented in long-term studies, particularly in real-world clinical settings. Selexipag, an oral selective prostacyclin IP receptor agonist, has demonstrated clinical and hemodynamic benefits in pulmonary arterial hypertension. Following its incorporation into routine clinical practice in Brazil, selexipag has been increasingly used in eligible patients with PAH-Sch. However, evidence regarding its long-term outcomes in this specific population is limited. PROPULSE-Sch is a single-center, observational, longitudinal study with an ambispective design, combining retrospective data and prospective follow-up. The study evaluates long-term clinical outcomes associated with exposure to selexipag in patients with PAH-Sch, compared with a mirror cohort of clinically similar patients who did not receive selexipag prior to its availability at the center. To reduce confounding and indication bias inherent to observational comparisons, analyses are conducted using propensity score matching. All treatment decisions, including initiation and intensification of therapy, are made exclusively by the treating physicians as part of routine clinical care. The study does not mandate any intervention, treatment assignment, or protocol-driven management. Data are obtained from medical records and standard follow-up visits. By using real-world data and robust observational methods, this study aims to contribute clinically relevant evidence on long-term outcomes in schistosomiasis-associated pulmonary arterial hypertension.