The European Respiratory Society Task Force, including a patient representative, used GRADE to update PAH treatment recommendations on two questions: sotatercept, an activin signalling inhibitor acting on a new pathway, and the role of repeat right heart catheterisation during follow-up.
It recommends add-on sotatercept for patients already on PAH drugs who remain at intermediate-low, intermediate-high or high risk of death, based on high-certainty evidence from randomised trials. It makes no recommendation for low-risk patients, who were few in the trials. Patients on sotatercept should be managed in pulmonary hypertension centres with regular follow-up. It suggests right heart catheterisation during follow-up for patients not at low risk when the result would change treatment.
For practice, this moves sotatercept into the standard escalation pathway for patients who are not achieving low-risk status on existing therapy. Sotatercept's regulatory status and cost in India are not addressed here and should be checked; access will be the main limit.
- Reassess risk status at every PAH follow-up using a validated multiparameter tool.
- Consider add-on sotatercept in patients on PAH drugs who remain at intermediate-low risk or worse.
- Refer patients for sotatercept to a pulmonary hypertension centre for initiation and monitoring.
- Monitor haemoglobin and platelets and watch for telangiectasia and bleeding on sotatercept.
- Repeat right heart catheterisation when the result would change treatment in patients not at low risk.
Why it matters
It formally adds a fourth treatment pathway to PAH escalation for patients not reaching low risk.
Don't overread it
No recommendation was made for low-risk patients, and access in India is not established.
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