To date sotatercept has only been studied in patients with prevalent pulmonary arterial hypertension receiving stable background therapy, not as part of the initial treatment regimen in newly diagnosed patients. A case report, published recently on the ERJ Open Research, describes a 25-year-old woman who developed severe high-risk idiopathic pulmonary arterial hypertension (IPAH) shortly after childbirth, presenting with near-complete haemodynamic collapse and requiring intensive care. Vasoreactivity testing was negative. After failing to tolerate intravenous treprostinil, sotatercept was added to her treatment regimen alongside macitentan and tadalafil. She improved rapidly, was discharged within weeks, and by July 2025 — just three months after diagnosis — her haemodynamics had nearly normalised and her NT-proBNP had returned to normal levels.
While the authors caution that this is a single case report, they note that clinical improvement began only after initiating sotatercept, suggesting the drug may have played a key role. The findings support the emerging concept of early combination therapy aimed at achieving remission in pulmonary arterial hypertension, and a prospective multicentre study is being planned to explore this further.
Read more at this link on ERJ Open Research
Citation
Park DH, Kamp JC, Seeliger B, Hoeper MM, Olsson KM. Initial triple combination therapy including sotatercept in a patient with severe idiopathic pulmonary arterial hypertension: a case report. ERJ Open Res. 2026 Mar 30;12(2):01209-2025. doi: 10.1183/23120541.01209-2025. PMID: 41918949; PMCID: PMC13034064.