European Respiratory Society clinical practice guidelines update for the treatment of pulmonary arterial hypertension
In brief
Add-on sotatercept advised for intermediate-to-high risk PAH patients
The updated ERS guideline gives a strong recommendation for adding sotatercept to existing therapy in pulmonary arterial hypertension patients judged at intermediate-low, intermediate-high, or high risk of death, based on high-certainty trial data. It suggests routine right-heart catheterisation during follow-up for these risk groups to guide treatment, while no guidance is offered for low-risk patients due to limited evidence.
- Journal
- The European respiratory journal (Q1)
- Published
- 7 September 2026
- Study design
- Randomized controlled trial
- Evidence level
- Level 1, High (CEBM 1b)
- Authors
- Gabor Kovacs, Katarina Zeder, Marc Humbert, Robin Condliffe, Ingrid Toews, Athenais Boucly, et al.
- PMID
- 42705705
- DOI
- 10.1183/13993003.01178-2026
Why clinicians should know about it
- Picked for Cardiology and Cardiovascular Medicine (top studies of the week, 13 September 2026): ERS guidelines update for PAH treatment
- Picked for Pulmonary and Respiratory Medicine (paper of the day, 10 September 2026): ERS guideline update for PAH treatment including sotatercept
Abstract
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.
Abstract as published, via PubMed.
For healthcare professionals. The summary is generated by AI from the published abstract, and the evidence level is assigned automatically from the study design on the Oxford CEBM hierarchy. Neither is medical advice. Read the full paper before changing practice.