1 Recommendations

1.1

Sotatercept, with other pulmonary arterial hypertension (PAH) treatments, can be used as an option to treat PAH in adults with World Health Organization functional class (WHO FC) 2 or 3, to improve exercise capacity. WHO FC 2 or 3 corresponds to low, intermediate–low or intermediate–high risk on the European Society of Cardiology and European Respiratory Society (ESC/ERS) risk-stratification framework. Sotatercept can be used if:

it is started when the PAH is at intermediate–low risk at follow up after usual treatment for PAH
the PAH has progressed to intermediate–high risk, if treatment with sotatercept was started when the PAH was intermediate–low risk.

Sotatercept can only be used if the company provides it according to the commercial arrangement.

1.2

This recommendation is not intended to affect treatment with sotatercept that was started in the NHS before this guidance was published. People having treatment outside this recommendation may continue without change to the funding arrangements in place for them before this guidance was published, until they and their NHS healthcare professional consider it appropriate to stop.

What this means in practice

Sotatercept must be funded in the NHS in England for the condition and population in the recommendations, if it is considered the most suitable treatment option. Early funding for sotatercept is available through the Innovative Medicines Fund from 14 May 2026 until 90 days after final publication of this guidance. After that, sotatercept must be funded through routine commissioning in England.

There is enough evidence to show that sotatercept provides benefits and value for money, so it can be used routinely across the NHS in this population.

NICE has produced tools and resources to support the implementation of this guidance.

Why the committee made these recommendations

Usual treatment for PAH in people with WHO FC 2 or 3 is a combination of endothelin receptor antagonists and phosphodiesterase type-5 inhibitors as background treatments, plus selexipag. Usual treatment for PAH in people with WHO FC 3 can also include prostaglandin I2 analogues. Sotatercept would be an alternative to selexipag.

WHO FC 2 or 3 corresponds to low, intermediate–low or intermediate–high risk on the ESC/ERS risk-stratification framework, which is used to classify risk in NHS practice.

The company asked for sotatercept to be considered only for people at ESC/ERS intermediate–low risk status at follow up when starting treatment. This does not include everyone it is licensed for. The company also proposed that people could continue sotatercept if they started treatment at the intermediate–low-risk status and their PAH progresses to intermediate–high risk.

Clinical trial evidence shows that sotatercept plus background treatments improves exercise capacity and WHO FC compared with placebo plus background treatments.

Sotatercept has not been directly compared in a clinical trial with selexipag. Indirect comparisons suggest that sotatercept is likely to work better, but the evidence is uncertain.

There are also uncertainties with some of the assumptions in the economic model, particularly around how it calculated the likelihood of people's conditions improving or getting worse, especially in the long term. But when considering the condition's severity, and sotatercept's effect on quality and length of life, the most likely cost-effectiveness estimate is within the range that NICE considers an acceptable use of NHS resources. So, sotatercept can be used.