Sotatercept to Wean Off Prostacyclin Infusion Therapy for Pulmonary Arterial Hypertension: A Case Report.

The activin signaling inhibitor sotatercept was approved for Group 1 pulmonary arterial hypertension (PAH) based on Phase 2 and 3 clinical trials showing significant improvements in primary outcomes; reduced pulmonary vascular resistance (PVR) and increased 6-min walk distance (6MWD), respectively. However, the efficacy and safety of transitioning off background therapies, including infusion prostacyclins, in patients receiving sotatercept are currently unknown. We report here a patient who was enrolled in sotatercept clinical trials (STELLAR/SOTERIA); during this period, he gradually transitioned from intravenous treprostinil. Subjective, physiologic, echocardiographic, and hemodynamic data after 2.5 years without intravenous therapy are presented. These results suggest that weaning off intravenous PGI2 may be feasible in some patients, but questions remain about the durability of the response and possible long-term adverse side effects.