How is pulmonary hypertension diagnosed and treated in children?

Pulmonary hypertension is usually diagnosed with a few different tests. Firstly, your child’s doctor may ask about their medical history, this might give clues to what has caused the symptoms.

Tests for pulmonary hypertension may include:

• An echocardiogram – this uses ultrasound to give more information about the structure of the heart and how the heart is working. It can also give some information about the blood pressure in the lung arteries.
• An electrocardiogram (ECG) – a test to measure your heartbeat rhythm and electrical activity.
• Exercise tests – this includes exercising while your heart rate, blood pressure and oxygen levels are monitored.
• Lung function tests – these tests measure how well your child’s lungs are working and how much air their lungs can hold.
• Chest X-ray – a picture of your child’s heart and lungs will help their doctor to see if there’s an underlying lung condition or if the heart is enlarged.
• Other imaging tests – (such as a CT or MRI scan). These can build up very detailed pictures of the heart and major blood vessels as well as give detailed images of the lungs if an underlying lung condition is suspected.
• Sleep study (Polysomnography PSG) – this will look for evidence of obstructive sleep apnoea (OSA) which can lead to PH.
• 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. This is done under general anaesthetic, so your child won’t be aware it’s happening.

If your child’s doctor thinks it is pulmonary hypertension, your child will be referred for further investigation by a specialist.

In the UK, there is a national specialist service for children run 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.

There is no cure for pulmonary hypertension, but the symptoms can usually be improved with the right treatment. It’s important for pulmonary hypertension to be treated as soon as possible because it can get worse over time.

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

The treatment for your child’s pulmonary hypertension will depend on the test results and how severe the disease is. These will all be discussed 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. A referral for lung transplantation may be discussed for severe pulmonary hypertension.

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

If your child has been diagnosed with PH, it’s important for them to continue exercising and take part in any activity they can. Speak to your health care professional about what activities your child can safely do.

It’s a good idea to let your child’s school know about their pulmonary hypertension, this is so that they can make any adjustments if needed. For example, making the school building easily accessible if your child can’t walk up stairs.

Ask your child’s doctors who you can contact if you have any questions, or if you’re unsure about something. It’s important to be able to recognise if your child’s condition is getting worse, or if they’re becoming ill, and when you should seek emergency care. Agree a plan with the doctor or nurse about what you should do.

What is the outlook for a child with PH?

The long-term outlook for PH is different for each child, and can depend on a few things:

• how severe your child’s PH is
• any other conditions they have
• their overall health and lifestyle
• how they respond to treatment.

Speak to your specialist team about your child’s individual situation.

Looking after a child with a long-term condition, like PH, can be difficult. It’s important you take time to take care of yourself, as well as your child. Talk to your doctor or nurse if you feel like you’re struggling to cope. You can also always call our helpline – they’re there to answer your questions, or just for a chat. Call 0300 222 5800, Monday to Friday between 9am – 5pm.

You may also want to join our Parent and Carer Support Network which provides support and a space for parents and carers of children with lung conditions to come together.

Pulmonary Hypertension Association UK (PHA UK) has more information and support for parents of children who have been diagnosed with pulmonary hypertension.