N Engl J Med 2021 04;384(13):1204-1215
From the Department of Respiratory and Intensive Care Medicine, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, INSERM Unité Mixte de Recherche 999, Université Paris-Saclay, Le Kremlin-Bicêtre, France (M.H., D.M.); the Division of Cardiovascular Medicine, Department of Internal Medicine, University of Michigan Health System, Ann Arbor (V.M.); the National Heart and Lung Institute, Imperial College London, and the National Pulmonary Hypertension Service, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London (J.S.R.G.); the Department of Medicine, George Washington University, Washington, DC (M.G.-M.); the Department of Respiratory Medicine, Hannover Medical School, and the German Center for Lung Research - both in Hannover, Germany (M.M.H., K.M.O.); the Division of Pulmonary, Critical Care and Sleep Medicine, Tufts Medical Center (I.R.P.), and the Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital (A.W.), Boston, and Acceleron Pharma, Cambridge (S.M., J.B., P.G.L., J.O.P.) - all in Massachusetts; the Pulmonary Division-Heart Institute, University of São Paulo Medical School, São Paulo (R.S.), and Complexo Hospitalar Santa Casa de Porto Alegre, Pulmonary Vascular Research Institute, Porto Alegre (G.M.) - both in Brazil; the Department of Cardiology, Centro de Investigación en Red en Enfermedades Cardiovasculares, Hospital Universitario 12 de Octubre, Universidad Complutense, Madrid (P.E.S.); Arizona Pulmonary Specialists, Phoenix (J.F.); and the Divisions of Pulmonary Sciences and Critical Care Medicine, and Cardiology, University of Colorado, Anschutz Medical Campus, Aurora (D.B.B.).
Background: Pulmonary arterial hypertension is characterized by pulmonary vascular remodeling, cellular proliferation, and poor long-term outcomes. Dysfunctional bone morphogenetic protein pathway signaling is associated with both hereditary and idiopathic subtypes. Sotatercept, a novel fusion protein, binds activins and growth differentiation factors in the attempt to restore balance between growth-promoting and growth-inhibiting signaling pathways. Read More