Clinical Trial Finder

Inclusion Criteria:

• - Documented diagnostic RHC at any time prior to screening confirming the diagnosis of WHO PAH Group 1 in any of the following subtypes: - Idiopathic PAH.

• - Heritable PAH.

• - Drug/toxin-induced PAH.

• - PAH associated with connective tissue disease.

• - PAH associated with simple, congenital systemic-to-pulmonary shunts at least 1 year following repair.

• - PAH classified as WHO FC I or symptomatic PAH classified as WHO FC II to IV.

• - On stable doses of background PAH therapy and diuretics (if applicable) for at least 90 days prior to screening.

Exclusion Criteria.

• - Diagnosis of PH WHO Groups 2, 3, 4, or 5.

• - Diagnosis of the following PAH Group 1 subtypes: - human immunodeficiency virus (HIV)-associated PAH.

• - PAH associated with portal hypertension.

• - schistosomiasis-associated PAH.

• - PAH with features of significant venous/capillary pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH) involvement.

• - Is on the waiting list for lung transplant.

• - Pregnant or breastfeeding women.

• - History of full or partial pneumonectomy.

• - Pulmonary function test (PFT) values of forced vital capacity (FVC) < 60% predicted at the screening visit or within 6 months prior to the screening visit.

• - Initiation of an exercise program for cardiopulmonary rehabilitation within 90 days prior to the screening visit or planned initiation during the study.

• - History of more than mild obstructive sleep apnea that is untreated.

• - Known history of portal hypertension or chronic liver disease, including hepatitis B and/or hepatitis C (with evidence of recent infection and/or active virus replication), defined as mild to severe hepatic impairment.

• - History of restrictive, constrictive, or congestive cardiomyopathy.

• - History of atrial septostomy within 180 days prior to the screening visit.

• - Personal or family history of long QT syndrome (LQTS) or sudden cardiac death.

• - Left ventricular ejection fraction (LVEF) < 45% on historical ECHO within 6 months prior to the screening visit.

• - Any symptomatic coronary disease events within 6 months prior to the screening visit.

• - Cerebrovascular accident within 3 months prior to the screening visit.

• - Significant (≥ 2+ regurgitation) mitral regurgitation or aortic regurgitation valvular disease, mitral stenosis and more than mild aortic valve stenosis.

• - Prior exposure to sotatercept or luspatercept or history of allergic or anaphylactic reaction or hypersensitivity to recombinant proteins or excipients in investigational product.

• - Received intravenous inotropes (e.g., dobutamine, dopamine, norepinephrine, vasopressin) within 30 days prior to the screening visit.

• - Currently enrolled in or have completed any other investigational product study within 30 days.

• - Weight at the screening is over 85 kg.

Arms

Experimental: Sotatercept

Participants on background PAH therapy will receive sotatercept subcutaneous (SC) injections at a starting dose of 0.3 mg/kg with a target dose of 0.7 mg/kg every 3 weeks up to 24 weeks. Thereafter, participants may choose to continue receiving the treatment until approval of sotatercept in Japan.

Interventions

Biological: - Sotatercept

SC injection at a starting dose of 0.3 mg/kg with a target dose of 0.7 mg/kg every 21 days plus background PAH therapy.