PubMed, Google Scholar, and Clinicaltrials.gov were searched from inception to June 8, 2023, using the keywords and MeSH terms: "Sotatercept," "ACE-011," "ActRIIA-IgG1," "ACTRIIA-Fc," "MK-7962," "RAP-011," "ActRIIA-IgG1Fc," "Activin receptor type IIA antagonist," and "Pulmonary hypertension." Studies were selected if they were (1) RCTs and (2) included patients with PAH randomized between Sotatercept and placebo. Here, ACVR2A is linked to pulmonary arterial hypertension.