A63-23 Evolving Management of Pulmonary Arterial Hypertension in the Era of Activin Signaling Inhibitor
Abstract
Patients with pulmonary arterial hypertension (PAH) require double or triple maximally tolerated combination therapy, yet progression persists in many cases. Sotatercept, a first-in-class activin signaling inhibitor, reverses pulmonary vascular remodeling and improves hemodynamic parameters. We report a case of connective tissue disease-associated PAH (CTD-PAH) where the addition of sotatercept allowed for the safe reduction of high-dose intravenous prostacyclin therapy while achieving clinical and hemodynamic improvement.
A 54-year-old female with history of CTD-PAH diagnosed in 2009 with pulmonary vascular resistance (PVR) of 15 wood unit (WU), managed with endothelial receptor antagonist (ambrisentan followed by macitentan) in combination with PDE5 inhibitor (sildenafil followed by tadalafil) and subcutaneous treprostinil. These treatments resulted in marked improvement (PVR=6.8 WU). In July 2016, her PAH was adequately controlled, allowing for a switch from treprostinil to oral selexipag (PVR=3.9 WU). By 2023 worsening symptoms lead to re-initiation of intravenous treprostinil (PVR=11.1 WU) gradually escalated to 135 ng/kg/min. Despite therapy, she experienced progressive right heart failure and was deemed ineligible for lung transplant. In early 2024, repeat hemodynamics revealed a rising PVR (8.4 WU) requiring initiation of subcutaneous sotatercept as a fourth agent. She developed severe abdominal pain and uncontrolled diarrhea prompting temporary discontinuation of…
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