The treatment landscape for pulmonary arterial hypertension (PAH) continues to evolve as evidence emerges for therapies targeting different pathways involved in disease progression.¹ At the ERS Congress 2026, Professor Gábor Kovács, co-chair of the ERS task force and first author of the updated guidelines, presented updates from the 2026 ERS clinical practice guidelines for the treatment of PAH, focusing specifically on medical therapy and the integration of activin signaling inhibitor therapy.¹

While the broader guideline document also evaluates the role of follow-up right heart catheterization (PICO 3 and PICO 4), Prof. Kovács’s conference presentation centered on the medical therapy questions and safety considerations:¹

• PICO 1: Should patients with PAH at intermediate-low, intermediate-high or high risk of death during follow-up who already receive PAH drug(s) be treated with activin signalling inhibitors (compared to not being treated with activin signalling inhibitors)?
• PICO 2: Should patients with PAH at low risk of death during follow-up who already receive PAH drug(s) be treated with activin signalling inhibitors (compared to not being treated with activin signalling inhibitors)?
• Narrative question: What is the safety of adding activin signalling inhibitor therapy to patients with PAH who already receive PAH drug(s)?

The 2026 update applies the GRADE framework to evaluate the certainty of evidence and establish recommendation strengths based on the balance of benefits and harms.¹ The presentation also detailed practical clinical considerations for safety monitoring with sotatercept, covering hematological, bleeding, telangiectatic, and cardiopulmonary complications.¹ In view of these considerations, patients receiving sotatercept should be carefully followed in specialized pulmonary hypertension centers, given the relatively limited clinical experience with the drug and the incomplete understanding of its mechanism of action in PAH.¹

The key medical therapy recommendations and treatment algorithm updates highlighted during the presentation are summarized in table 1.¹

Table 1. Key recommendations from the 2026 ERS clinical practice guidelines update for the treatment of PAH as presented by Professor Kovács

ERA: Endothelin receptor antagonist; ERS: European Respiratory Society; I/H/D-PAH: Idiopathic, heritable or drug-associated pulmonary arterial hypertension; IV: Intravenous; PAH: Pulmonary arterial hypertension; PAH-CTD: Pulmonary arterial hypertension associated with connective tissue disease; PCA: Prostacyclin analogue; PDE5-i: Phosphodiesterase type 5 inhibitor; RCT: Randomized controlled trial; SC: Subcutaneous

In summary, the 2026 ERS clinical practice guidelines strengthen the role of sotatercept within a risk-based treatment strategy for PAH, with a strong recommendation for patients who remain at intermediate-low, intermediate-high or high risk of death despite PAH drug therapy.¹ No recommendation was made for patients who remain at low risk due to the low certainty of available evidence, and treatment decisions should be individualized.¹ Overall, the update reinforces a risk-based, reassessment-driven approach to PAH management, with ongoing evaluation of treatment response guiding further treatment optimization.¹