Emphasis Autumn/Winter 2022

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Promising results for new PAH drug A new medication, that could be approved for use next year, has produced promising results in its latest clinical trial. Using six-minute walk tests, the STELLAR trial showed that when sotatercept is given in addition to other therapies, it significantly improves exercise capacity in people with pulmonary arterial hypertension. The drug, which is given under the skin as a subcutaneous injection, is the first of its kind to rebalance what’s known as the ‘bone morphogenic protein pathway’. While existing treatments are designed to help manage the symptoms of PH, sotatercept is the first to target the root cause of the disease. Neil Hamilton, Consultant Pharmacist in PH, told us: “Sotatercept is an exciting new treatment which works in a different way to any of the current medicines we can prescribe. We are optimistic about sotatercept because the results from early clinical trials have been very positive. More evidence of effectiveness and safety is needed, so further studies are underway. These international trials will involve large numbers of patients and if these prove successful, sotatercept could be approved by regulatory bodies next year. As such, it is not currently available to anyone outside clinical trials. Sotatercept may not be suitable for everyone with PH,

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but expert centres in the UK are recruiting patients to these trials – so if you are eligible, your centre would discuss your participation at your next clinic visit.” Online reaction to the results of the STELLAR trial: "This is a big deal in pulmonary hypertension”

Dr Mark Toshner (@mark_toshner)

“This might be the most exciting advancement in pulmonary arterial hypertension since I started practice” Dr Jason Weatherald (@AlbertaPHdoc)

Blood sample research may help identify the best treatment for CTEPH A study by scientists at Royal Papworth Hospital could lead to doctors being able to identify which patients with chronic thromboembolic pulmonary hypertension (CTEPH) would be best suited to pulmonary endarterectomy surgery. The surgery, knows as a PEA, removes scars made by old blood clots in the pulmonary arteries and it is potentially curative. However, some may be left with residual PH, and as a major and complex operation, it is only suitable for approximately 60-70% of patients.

Researchers at Royal Papworth Hospital have studied blood samples from patients before and after this surgery, to identify changes in chemicals contained within the blood. The study, titled Plasma metabolomics exhibit response to therapy in chronic thromboembolic pulmonary hypertension, has shown differences between healthy individuals and patients with CTEPH, including changes correlating with the severity of the disease. A small number of the chemicals were also found to be different from those in patients with idiopathic pulmonary arterial hypertension (IPAH), which may lead to a new a way of identifying the type of PH a person has. Ultimately, those behind the study believe that chemicals that are either produced, used or changed by the heart and lungs may eventually be used to identify which patients are most suitable for PEA surgery to treat CTEPH. The researchers would like to thank all the patients who took part in this important study.

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