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Pulmonary hypertension in children

How is pulmonary hypertension diagnosed and treated in children?

How is pulmonary hypertension diagnosed in children?

The doctor will examine your child and ask about their medical history. This may give clues to the underlying cause. Your child will have tests to look at the way the heart and major blood vessels in the body are functioning.

Your child will probably have a scan called an echocardiogram. This uses ultrasound to give more information about how the heart is working. It can also give some information about the pressure in the pulmonary arteries.

As well as repeating the echocardiogram, your child may have additional tests to diagnose pulmonary hypertension including:

  • an electrocardiogram (ECG) to check their heart
  • an exercise test.

If pulmonary hypertension is suspected, your child will be referred for further investigation and management by a specialist. In the UK, there is a national specialist service for children co-ordinated by Great Ormond Street Hospital for Children NHS Foundation Trust. Children may be seen at Great Ormond Street Hospital or a regional paediatric cardiology centre with close links to the main national centre.

They may also need other tests to confirm the diagnosis or help plan treatment. These may include:

  • imaging tests (such as a CT or MRI scan). These can build up very detailed pictures of the heart and major blood vessels
  • cardiac catheterisation. This is performed by a children’s heart specialist. A thin, flexible tube is passed into a large vein in the neck or groin and pushed through to the pulmonary artery, to measure blood pressure in the major blood vessels from the heart to the lungs.

Telling other health professionals

It’s very important to tell other health professionals caring for your child (including their dentist) about their diagnosis of pulmonary hypertension. It’s especially important if they need a general anaesthetic. This can be very dangerous for people with pulmonary hypertension and specialist advice is needed.

What is the treatment for pulmonary hypertension?

If your child has another condition that is causing pulmonary hypertension (for example lung disease or a leaky heart valve), doctors will focus on treating this first. This will help your child’s symptoms.

There is no cure for pulmonary hypertension, but the symptoms can usually be improved with appropriate treatment. Because pulmonary hypertension gets worse over time (placing an increasing strain on the heart), it’s important for it to be treated as soon as possible.

The most appropriate treatment for your child’s pulmonary hypertension will depend on the outcome of tests and how severe the disease is. These will all be discussed fully by your specialist team, but may include:

  • medication to relax the blood vessels in the lungs, to encourage new blood vessels to grow, or to stop blood clots from forming
  • water tablets (diuretics), which can help reduce swollen ankles
  • oxygen therapy, to increase the levels of oxygen in the blood, as this may help the blood vessels in the lung
  • surgery to relieve the strain on the heart. For severe pulmonary hypertension a referral for lung transplantation may be discussed.

If your child is diagnosed with pulmonary hypertension, they will receive specialist care at a pulmonary hypertension treatment centre.

Day-to-day life

After diagnosis, it’s good for your child to keep participating in normal activities where possible. Make sure the school is informed about your child’s pulmonary hypertension, and tell them if any adjustments need to be made for them.

Preventing infection

It’s important to make sure your child’s immunisations are up-to-date, including the flu jab. This will help to prevent infections, which could stop your child getting enough oxygen.

Where can I get more information and support?

Pulmonary Hypertension Association UK (PHA UK) has information and support for people affected by pulmonary hypertension. This includes information on children and PH.

We have information on pulmonary hypertension in adults.

We use your comments to improve our information. We cannot reply to comments left on this form. If you have health concerns or need clinical advice, call our helpline on 03000 030 555 between 9am and 5pm on a weekday or email them.

Last medically reviewed: September 2019. Due for review: September 2022

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.