Treatment for pulmonary hypertension

Pulmonary hypertension cannot usually be cured. But there are treatments that can help reduce your symptoms and help you to manage your condition. It’s important to treat pulmonary hypertension to stop it getting worse.

You will be treated by 1 of 8 specialist centres in the UK. Your treatment will depend on what group it is, what’s causing it and how severe it is. The Pulmonary Hypertension Association (UK) has more detailed information about individual treatments for pulmonary hypertension.

Your specialist will offer you treatments called pulmonary vasodilators. They aim to lower the blood pressure in your lungs by relaxing and opening up the pulmonary arteries. With more space for the blood to pass through, the right side of your heart is under less strain and should work better. This means that your symptoms should improve. Pulmonary vasodilators include medications called:

• phosphodiesterase 5 (PDE5) inhibitors
• endothelin receptor antagonists
• soluble guanylate cyclase stimulators (SGCSs)
• prostanoids.

The choice of medication is complex, and your specialist will discuss this with you. Other treatments help with your symptoms, called background (or supportive) therapy.

They may include:

• Diuretics or water tablets: these remove excess fluid from your body. They treat symptoms like swollen ankles.
• Oxygen therapy: this will increase the level of oxygen in your blood and may make you feel less breathless.
• Pulmonary rehabilitation (PR): PR is a programme of exercise and advice to help cope with breathlessness. Take a look at the PHA-UK Lifestyle site, which has more information on physical activity when living with pulmonary hypertension.

Your specialist will advise you:

• not to become pregnant
• not to undergo general anaesthesia for surgery unless absolutely necessary
• not to travel to high altitude places (above 1500-2000m)
• to keep up to date with flu and pneumonia vaccinations.

You may also need oxygen when flying – ask your health care professional about a fitness-to-fly test.

In both groups, pulmonary hypertension is a secondary condition. This means it’s caused by a heart or another lung condition - the primary condition. Examples of primary lung conditions are COPD, interstitial lung disease such as pulmonary fibrosis, and OSA.

If your pulmonary hypertension is a secondary condition, your health care professional will focus on treating the primary condition. If the primary condition improves with treatment, it should improve your pulmonary hypertension too. It’s very unlikely that you’ll be treated with pulmonary vasodilators if pulmonary hypertension is not your primary condition.

If your pulmonary hypertension is caused by blood clots, your specialist will treat you with anticoagulant medicine. This medicine stops more blood clots forming.

You might be offered:

• warfarin, which is taken as a tablet
• a group of anticoagulation medicines called NOACs (novel oral anticoagulants) or DOACs (direct oral anticoagulants). These are now used more often than warfarin, but warfarin is still needed in some cases.

If your blood clot has caused scar tissue in your pulmonary arteries, you may be offered a pulmonary endarterectomy (PEA). This is an operation to remove scar tissue from the inside layer of the pulmonary arteries. This improves the blood flow and reduces the pressure inside the arteries.

If a pulmonary endarterectomy isn’t suitable, you may be offered a new procedure called balloon pulmonary angioplasty (BPA). In this procedure, a tiny balloon is guided into a narrowed lung artery and inflated for a few seconds to widen the artery. This can be done several times, and in different parts of the lung during one procedure. The aim is to improve blood flow through the lungs and reduce pressure on the heart. Royal Papworth Hospital, where BPA procedures are performed, have said that early use of this technique has showed BPA can lower blood pressure in the lung arteries, improve breathing and allow you to exercise more.

Pulmonary endarterectomy and balloon pulmonary angioplasty are specialist procedures currently only performed in the UK at the Royal Papworth Hospital.

If you’re not suitable for surgery or you still have some pulmonary hypertension after the operation, you may be offered a drug that is a soluble guanylate cyclase stimulator (SGCS), which has recently been found to be effective in slowing the progression of CTEPH. You may also be given another pulmonary vasodilator, or a combination of tablets.

Because pulmonary hypertension in this group is caused by a range of different factors, there’s no standardised treatment. Your specialist will discuss the best treatment for you.

Research has shown there are few studies investigating the treatment of group 5 PH, plus the studies involve a limited number of people. This means that treatment for group 5 PH is often guided by smaller studies and individual case reports.

Transplant surgery

If your pulmonary hypertension doesn’t respond to treatment, a double lung or a heart-lung transplant might be an option.

Clinical trials

Researchers are constantly working on new drugs to help treat pulmonary hypertension. Clinical trials look at how effective these new drugs are and are an important part of the research. Pulmonary hypertension is a relatively rare disease, so it can be difficult to find enough people for a trial. Clinical trials are usually available at specialist centres, so you may be asked if you’d be willing to take part. Read more about clinical trials for pulmonary hypertension on the PHA UK website.

The Pulmonary Hypertension Association (UK) provides support and detailed information for people affected by pulmonary hypertension.