Pulmonary hypertension

Treatment for pulmonary hypertension

It’s important to treat pulmonary hypertension to stop it getting worse. Your treatment will depend on what type it is and what’s causing it.

Type 1: Pulmonary arterial hypertension (PAH)

If you have pulmonary arterial hypertension, you’ll be referred to a specialist centre for treatment.

You’ll usually have background therapy first – treatments to help with your symptoms.

Background therapy may include:

  • diuretics: These remove excess fluid from your body. They treat symptoms like swollen ankles.
  • oxygen therapy: In oxygen therapy, you breathe air with a higher concentration of oxygen than normal air. This increases your oxygen levels and improves your symptoms.
  • anticoagulation: With some forms of PAH you have a greater risk of getting blood clots. Anticoagulant medicines like warfarin stop blood clots forming.
  • pulmonary rehabilitation: This is a programme of gentle physical exercise and advice, which helps you cope with breathlessness. The classes are run by physiotherapists and specialist nurses.

Your health care professional may also offer you treatments called pulmonary vasodilators. These 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 can be taken as tablets, with a nebuliser or as an injection. You can find out more about these treatments on the Pulmonary Hypertension Association website.

Your health care professional will advise you:

  • not to become pregnant
  • not to travel to high altitude. This includes avoiding high altitude destinations like mountains, as well as flying
  • to keep up to date with flu and pneumonia vaccinations

Types 2 and 3: Pulmonary hypertension due to left heart disease or lung conditions

In both these cases pulmonary hypertension is a secondary condition. This means it’s caused by another lung or heart condition, which is the primary condition.

If your pulmonary hypertension is a secondary condition, your health care professional will focus on treating the primary condition. If you get the best treatment for your primary condition, it should improve your pulmonary hypertension too.

In these cases, it’s very unlikely that you’ll be treated directly for pulmonary hypertension.

Type 4: Pulmonary hypertension due to blood clots (chronic thromboembolic pulmonary hypertension, CTEPH)

If your pulmonary hypertension is caused by blood clots, your health care professional will treat you with anticoagulant medicine. This medicine stops more blood clots from forming. The most common anticoagulant medicine is warfarin, which is taken as a tablet.

If your blood clot has caused scar tissue in your pulmonary arteries, you may be offered a pulmonary endarterectomy. 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. This is a specialist operation and is only performed at Papworth Hospital in Cambridgeshire.

If you’re not suitable for surgery or you still have some pulmonary hypertension after the operation, you may be given tablets called pulmonary vasodilators. These help to open up narrowed pulmonary arteries.

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 health care professional will decide the best treatment for you, which may include some of the treatments above.

Other treatments

Lung transplant

If your pulmonary hypertension doesn’t respond to treatment, a lung transplant might be an option. This is a very rare procedure and not everyone is suitable for a lung transplant. Factors such as age and other medical problems would be considered, as well as the suitability of donors.

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. Clinical trials are nearly always available at specialist centres and you may be asked if you want to take part.

Further information

The Pulmonary Hypertension Association (UK) provides support and information for all people affected by pulmonary hypertension. Go to their website or call 01709 761450.

You can also call our helpline for support and advice on living with a lung condition. Find out how to get in touch.

Download our pulmonary hypertension PDF (342KB)

Last medically reviewed: January 2016. Due for review: January 2019

This information uses the best available medical evidence and was produced with the support of people living with lung conditions. Find out how we produce our information. If you’d like to see our references get in touch.