Why the study?
Pulmonary arterial hypertension is a severe, progressive disease, prompting updated evidence-based treatment recommendations focusing on sotatercept and the role of right heart catheterisation during follow-up.
Does add-on sotatercept and right heart catheterisation during follow-up improve management in patients with pulmonary arterial hypertension?
Does add-on sotatercept and right heart catheterisation during follow-up improve management in patients with pulmonary arterial hypertension?
Updated ERS guidelines recommend sotatercept as add-on therapy and right heart catheterization during follow-up for PAH patients at intermediate-to-high risk of death.
Answers are grounded in this paper and cite their sources.
Captured external expert commentary on this paper, strongest first. Original sources are linked where available.
“Since the number of low-risk patients included in clinical trials was much smaller, the working group was unable to make a recommendation on this issue. This does not mean that this treatment should not be administered or that it should be administered routinely. Treatment decisions for these patients must be made on a case-by-case basis, after considering all available clinical data.”
“Administered subcutaneously every 3 weeks, sotatercept is now classified in the 2026 update of the guidelines as a second-line treatment for patients at intermediate or high risk who are already receiving oral therapy and who have not achieved low-risk status. For low-risk patients, this should be discussed on a case-by-case basis.”
Endorses sotatercept add-on for intermediate- to high-risk PAH on background therapy; extends RCT data into updated guideline-directed care.
Background Pulmonary arterial hypertension (PAH) is a severe, progressive disease of the small pulmonary arteries. These guidelines provide updated evidence-based recommendations for the treatment of PAH focusing on the latest scientific evidence on sotatercept, an activin signalling inhibitor targeting a novel pathway for the treatment of PAH, and on the role of right heart catheterisation (RHC) in the management of PAH patients during follow-up. Methods A European Respiratory Society (ERS) Task Force, comprising global experts, methodologists and a patient representative, developed these clinical practice guidelines in accordance with ERS methodology and the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) approach. Four PICO (Patients, Intervention, Comparator, Outcomes) questions and one narrative question have been addressed. Recommendations The Task Force recommends add-on treatment with sotatercept in PAH patients who already receive PAH drugs and are at intermediate-low, intermediate-high or high risk of death during follow-up; this recommendation is based on a high certainty of evidence available from randomised controlled trials (RCTs). In PAH patients at low risk of death, the Task Force does not provide a recommendation due to the low certainty of evidence and the limited number of such patients included in RCTs. To monitor safety and efficacy, patients receiving sotatercept should be treated in pulmonary hypertension centres and their management should include regular follow-up and appropriate examinations. To guide treatment strategy, the Task Force suggests performing RHC during follow-up in PAH patients who receive PAH drugs and are at intermediate-low, intermediate-high or high risk of death, when therapeutic consequences are expected. The Task Force does not provide a recommendation for this question in PAH patients at low risk of death, due to the very low certainty of evidence. Conclusion The ERS guidelines on the treatment of PAH provide an updated evidence-based framework for the optimal management of patients with PAH.
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Kovács et al. (2026) conducted a review in Pulmonary arterial hypertension (PAH). Sotatercept and right heart catheterisation (RHC) was evaluated.
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