The pulmonary hypertension service at Royal Brompton Hospital is run by the Royal Brompton pulmonary vascular unit. The service is one of seven centres that make up the UK’s national pulmonary hypertension service.

What is pulmonary hypertension?

Pulmonary hypertension (PH) refers to a group of conditions where the blood pressure in the lungs is raised due to narrowing of blood vessels in the lungs. It is a rare condition that affects approximately one in 20,000 people in the UK, unlike raised blood pressure (systemic hypertension), which is more common.

It is important to correctly diagnose, treat and manage pulmonary hypertension because it is a potentially a serious life-threatening condition.PH can occur with or without an identifiable or known cause.

The most common symptoms of PF are shortness of breath and fatigue. Less common symptoms include:

  • dizziness
  • fainting (syncope)
  • swelling of feet or ankles
  • palpitations
  • chest pain, particularly on exertion
  • cough.

Get an appointment

To arrange an appointment at the hospital, you must be:

  • entitled to NHS care
  • registered with a GP in the UK.

We will need a referral letter from your GP or hospital consultant. This can be sent by fax or post. It is important that your GP includes copies of all recent investigations or assessments.

Your first appointment

The first appointment may be at one of PH outpatient clinics held every:

  • Tuesday (am)
  • Thursday (pm)
  • Friday (am)

At the appointment, you will meet the PH team and might have some initial tests, including:

You will also have the opportunity to talk to staff providing psychological and social support. The PH outpatient clinic provides on-going support and treatment for existing patients.

Where are the clinics?

Clinics are located in Fulham Road (outpatients east or west) on the ground floor. Blood tests, chest x-rays and ECG are in the same building.

Your echo may be done in the Sydney Street building. A porter with a wheelchair can help if you cannot walk that far.

If you have not received an echo appointment, contact us on 0207 351 8362 to confirm whether you need one.

Further assessment and investigation

After your first appointment, we may ask you to return as an inpatient for three to five days so we can check your health needs and treatment options. This may include a number of tests to assess your health.

You will discuss this plan in clinic with one of the consultants or specialist nurses.

The admissions coordinator will call you to arrange when this is convenient for you and discuss any logistical aspects.

You can discuss any clinical aspects with the clinical nurse specialist before you come in, by:

  • ringing the PH office
  • email.

On the day of the appointment, you should bring your usual day clothes to wear. It is useful to have someone with you when the diagnosis and proposed treatment options are discussed, so you may want to talk to the team about the timing of these discussions.

Occasionally we may need to admit you urgently for further tests or treatment. Your care will then take place on one of the main hospital wards, usually Paul Wood or York ward in the Sydney Street building, under the care of the pulmonary hypertension team.

In certain circumstances, patients have agreed for their results to be used for research. This will only happen if we receive your written permission.

Scans and tests

Scans and tests that may be carried out to assess your health include:

  • lung function tests: a blowing test to assess the size, capacity and gas transfer ability of your lungs
  • walk test: a test to see how far you can walk in six minutes
  • electrocardiogram (ECG): a simple test to check heart rhythm and electrical activity
  • echocardiogram: an ultrasound scan of your heart
  • computed tomography (CT) lung scan: to look at the structure of the lungs and blood vessels
  • right-heart catheter study: measures blood pressures within your heart and lungs
  • cardiopulmonary exercise test (CPET): to assess how your lungs, heart and muscles react together when you exercise on a bike
  • some patients may also undergo an exercise right heart catheterisation with Dr Colm McCabe to explain exercise-induced symptoms
  • magnetic resonance imaging (MRI) scan: to assess cardiac anatomy and determine blockages in lung vessels - his can be a noisy scan and will not take place if you have any metal in your body.

Some of these tests may already have been performed at your local hospital and will only be repeated if necessary.

The results of the tests will be explained to you. There may also be other procedures depending on your situation.

We sometimes also ask patients to see Dr James Hull, who runs the unexplained breathlessness service and performs detailed supervised cardiopulmonary exercise tests with arterial blood gas analysis in the Fulham Road wing of Royal Brompton Hospital, and has a team of experts who deal with patients with breathlessness.

If a diagnosis of pulmonary hypertension is confirmed you will meet one of the pulmonary hypertension specialist nurses and be provided with information from the Pulmonary Hypertension Association (PHA). 

Treatment

Further assessment and investigation will help us to decide if you need treatment with specialist drugs. Most patients who receive treatment will be patients with pulmonary arterial hypertension (PAH). 

PAH is a type of PH when the blood vessels before the lung capillary bed are affected, and pressures are high in these bloods vessels. Being put into this group will depend on your baseline tests. Your treatment will be:

  • supported by the latest scientific evidence
  • available for routine use on the NHS to treat pulmonary hypertension
  • dependent on your local primary care trust agreeing to fund this therapy.

Most patients who need treatment are started on tablets, but some people will start more complicated treatments due to the nature and severity of their condition.

The funding for these special treatments is, which means your GP does not pay for them.

Homecare, a company linked to our pharmacy team, will deliver the medicine directly to you.

If you have any problems or run out of medicine, contact the pharmacy team well in advance by calling 0207 352 4199.

Read PHA information about drug treatment options: 

Clinical drug trials 

You may also be given information about clinical drug trials that you might be able to enroll in. The PH team is actively involved in multi-centre studies, which can provide opportunities not available with usual clinical treatments.

You are under no pressure to participate and it will not affect your ongoing care if you decide you do not wish to participate.

If you need a referral to another specialist for further treatment, we will discuss this with you.

Pulmonary endarterectomy

Some patients with a subgroup of pulmonary hypertension due to the effects of chronic blood clots, chronic thromboembolic disease (CTEPH), will undergo assessment for pulmonary endarterectomy (PEA), which in the UK is performed at Papworth Hospital near Cambridge.

We work closely with the team at Papworth to enable appropriately patients to be able to access this important service. 

Papworth Hospital has published helpful information leaflets for patients:

We also work closely with the transplant teams at Harefield, Papworth and Newcastle Hospitals, as lung transplantation is a curative option for some patients.

Follow-up care

Your GP or consultant who referred you will usually be responsible for your long-term support and we may ask you to return to clinic for a check-up at regular intervals.

Intervals can be three, six or 12 months and will depend on your treatment and care plan.

For example, if you are started on PAH drug therapies, you will need a follow up usually three months after therapies are started or doses changed. 

This will usually include:

  • clinic review
  • six-minute walk
  • echo
  • blood tests.

The same model applies in our shared care centres and some patients will not need further follow-up.

If you get acutely unwell at home, contact your GP. They can then contact us for further advice regarding your care.

Echocardiogram (echo)

An echocardiogram, also known as an echo, is a test that uses sound waves to build up a moving picture of the heart.

ECG Holter monitor

This test monitors your heart rhythm over 24, 48 or 72 hours, or five or seven days. The monitor is about the size of a mobile phone and you will need to wear it around your waist or carr...

Consultants

Clinical nurse specialists

  • Carl Harries
  • Joana Barbosa
  • Helen Moorshead

Contact

Contact the PH team on 0207 351 8362 for:

  • more information about your treatment
  • general enquiries.

The phone is manned from 9am to 5pm, Monday to Friday. Any messages will be picked up if you can’t speak to someone immediately.

Further information

A very useful resource for patients, and an invaluable source of support, is the pulmonary hypertension association (PHA). Patients are encouraged to join this patient organisation.

Listen to this podcast from Dr Wort, as he explains the condition and the work the Trust is doing to treat patients. 

Visit the PHA website for more information and read these patient leaflets about:

What is pulmonary hypertension - Royal Brompton Hospital - August 2014 (pdf, 637KB)


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