treatment for Pulmonary Hypertension

Selexipag

Selexipag is the first medicine of a new class of treatments called prostanoids receptor agonists. It makes its beneficial effects by working in a similar way to other prostanoids. However, it has been designed to reduce the amount and severity of systemic (whole body) side effects caused by medicines such as iloprost or epoprostenol.

How to take it

Selexipag is a tablet which is taken twice a day, ideally with food. The tablets must be swallowed whole, not chewed or crushed. The appropriate dose varies between patients, in the same way as intravenous prostanoids. For this reason, everyone starts on a very low dose, which is increased slowly over a few weeks until the maximum tolerated dose is reached. Whilst this “uptitration” can be done at home, there is regular contact between patients and their PH centre to ensure this process is carried out safely. Patients are encouraged to increase the dose to the point where some side effects are experienced, then be reviewed. Selexipag tablets come in a variety of strengths so patients can easily up-titrate their dose, until the appropriate strength can be prescribed as one tablet twice a day.

Do not take selexipag if you:
Have liver problems
• Have severe coronary heart disease or unstable angina.
• Have had a myocardial infarction within the last six months.
• Have had a cerebrovascular events (e.g. transient ischaemic attack, stroke) within the last three months.
• Have severe arrhythmias.
• Have decompensated cardiac failure that is not under close medical supervision.
• Have congenital or acquired valvular defects with clinically relevant myocardial function disorders not related to pulmonary hypertension.
• Are pregnant or plan to become pregnant. It is not known if selexipag will harm your unborn baby.
• Are breastfeeding or plan to breastfeed. It is not known if selexipag passes into your breast milk. You and your doctor should decide if you would take selexipag or breastfeed. You should not do both.

Side effects

The most common side effects are:
• Headache.
• Nausea and vomiting.
• Pain in arms or legs.
• Low red blood cell count.
• Diarrhoea.
• Muscle and joint pain.
• Jaw pain.
• Temporary reddening of the skin (flushing).
• Reduced appetite.
• Rash on.

Other useful information

Selexipag is only available on prescription and should only be initiated by a specialist PH centre. If a dose is missed, patients should take a missed dose as soon as possible unless the next dose is within the next six hours. However, if treatment is missed for more than three days, restart selexipag at a lower dose and contact your PH centre for advice.

Last medically reviewed: December, 2018 • Due for review: December, 2021