Treatment for pulmonary hypertension
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 and 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 detail about individual treatments.
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)
The choice of medication is complex and your specialist will discuss this with you.
Other treatments will 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.
- anticoagulation: With some forms of PAH you have a greater risk of getting blood clots. Anticoagulant medicines like warfarin help prevent blood clots forming.
- pulmonary rehabilitation or PR: PR is a programme of exercise and advice to help cope with breathlessness.
Your specialist will advise you:
- not to become pregnant
- not to travel to high altitude (above 1500-2000m). You may also need oxygen when flying – ask your health care professional about a fitness-to-fly test
- to keep up to date with flu and pneumonia vaccinations
Types 2 and 3: Pulmonary hypertension caused by left heart disease or lung conditions
In both groups, pulmonary hypertension is a secondary condition. This means it’s caused by another lung or heart 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.
Type 4: Pulmonary hypertension caused by blood clots (chronic thromboembolic pulmonary hypertension, CTEPH)
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 new group of medicines called NOACs (novel oral anticoagulants) or DOACs (direct oral anticoagulants)
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. 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. Early use of this technique has showed it can lower blood pressure in the lung arteries, improve breathing and allow you to exercise more.
Both are specialist procedures currently performed in the UK only at 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.
Type 5: Pulmonary hypertension due to a range of causes
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.
If your pulmonary hypertension doesn’t respond to treatment, a double lung or a heart-lung transplant might be an option.
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.
The Pulmonary Hypertension Association (UK) provides support and detailed information for people affected by pulmonary hypertension.
You can also call our helpline for support and advice on living with a lung condition.
Pulmonary rehabilitation (PR)
Pulmonary rehabilitation is a physical exercise programme, designed for people with lung conditions and tailored for you.