Suneesh C. Anand, Muhammad Furqan, Adriano R. Tonelli, Daniela Brady, Avi Levine, Erika B. Rosenzweig, William H. Frishman, Wilbert S. Aronow, Gregg M. Lanier
Hillcrest Hospital and Cleveland Clinic. New York Medical College, Maria Fareri Children’s Hospital of Westchester and Westchester Medical Center.
United States
Cardiology in Review
Cardiol Rev 2025;
DOI: 10.1097/CRD.0000000000000837
Abstract
Pulmonary arterial hypertension (PAH) is a progressive disease characterized by proliferative remodeling and obliterative narrowing of the pulmonary vasculature. While outcomes have improved with existing treatments targeting 3 main pathways, there remains a critical need for novel therapies that address different and novel mechanisms of PAH. Sotatercept, recently Food and Drug Administration (FDA) approved, is a groundbreaking fusion protein that binds to activin and growth differentiation factors, rebalancing antiproliferative and pro-proliferative signals to reverse remodeling in both the pulmonary vasculature and the right ventricle. This review highlights current evidence exploring the safety and efficacy of sotatercept in the 2 landmark trials, phase 2 Pulmonary Arterial Hypertension and Sotatercept Trial and Research and phase 3 Sotatercept Treatment in Expansion of Long-term Learning and Assessment in PAH trial, which were instrumental in securing FDA approval for adult PAH patients with WHO functional class II or III symptoms already receiving background pulmonary hypertension therapy. Overall, sotatercept represents a landmark advancement in PAH treatment, offering hope for patients and the potential to delay or avoid lung transplantation. Importantly, this marks the beginning of an era of targeted therapies aimed at reverse remodeling in PAH while improving outcomes.
Category
Medical Therapy. Efficacy or Lack of Efficacy
Medical Therapy. Adverse Effects or Lack of Adverse Effects
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No