What is pulmonary hypertension?

Pulmonary hypertension refers to a group of conditions whereby the blood pressure in the lungs is raised due to narrowing of the lung blood vessels. It is a rare condition that affects approximately 1 in 20,000 people in the UK unlike the common "raised blood pressure" also known as systemic hypertension. It is important to correctly diagnose, treat and manage pulmonary hypertension because it is a potentially a serious life threatening condition.

Common symptoms

More common
Shortness of breath

Less common
Fainting (syncope)
Swelling of feet or ankles
Chest pain, particularly on exertion


Pulmonary hypertension has a large number of different causes but these can be summarised into five main groups below. The investigations performed by our specialist multidisciplinary team aims to identify the underlying cause for the pulmonary hypertension.

  1. Pulmonary arterial hypertension
    1. Idiopathic (unknown cause)
    2. Familial (inherited)
    3. Associated with connective tissue disease e.g. systemic sclerosis
    4. Associated with congential heart disease (patients born with a heart defect)
    5. Associated with liver disease e.g. cirrhosis
    6. Associated with HIV infection
  2. Pulmonary hypertension due to left heart disease
  3. Pulmonary hypertension due to lung disease or low oxygen levels
  4. Chronic thromboembolic pulmonary hypertension (chronic blood clots)
  5. Pulmonary hypertension due to miscellaneous condition

New Patients

How to obtain an appointment?

You will need a referral letter from your hospital consultant or GP. Your doctor can post or email the letter together with copies of recent investigations. If appropriate we will then arrange to see you at Papworth Hospital in approximately 4-6 weeks.

What happens next

New patients to the PVDU may be seen on Thoracic Day Ward (day visit only) or admitted as an inpatient to Chest Medical Unit depending on the required investigations, how unwell they are and their accessibility to Papworth Hospital. If you are waiting to attend the pulmonary vascular diseases service at Papworth Hospital and wish to ask a question about your admission you should phone one of the service administrators who will be happy to advise you. If you are to be admitted to hospital for a few days you should bring your ordinary clothes with you as well as normal nightclothes and toiletries. Please remember to bring all medication that you are taking in its original, labelled container.

Several tests and procedures will be performed in order to assess the type and severity of pulmonary hypertension, including:

  • Chest X-ray
  • Lung function test - blowing test to assess the size and capacity of your lungs
  • Walk test - a test to see how far you can walk in six minutes
  • ECG - electrical (heart tracing)
  • Echocardiogram - sonar scan of your heart
  • Blood tests
  • 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

Some of these tests may already have been performed at your local hospital and will only be repeated if necessary. There may also be other procedures depending on individual circumstances. The results of the tests will be explained to you. If we confirm a diagnosis of pulmonary hypertension you will meet one of our pulmonary hypertension specialist nurses and be provided with written information from the Pulmonary Hypertension Association ("Understanding pulmonary hypertension – information for patients").

Our patients tell us that it is helpful to have someone with them when we discuss the diagnosis and proposed treatment options.

Treatment options

In the last 10 years there have been a number of new treatments licensed for the treatment of pulmonary arterial hypertension. Importantly the treatment that we use is:

  1. Supported by clinical trial evidence
  2. Funded by the NHS routinely

The results of the tests allow us to work out whether you will benefit from specialist drugs. The treatment options available to you will be discussed including the "pros" and "cons" for each option. The majority of patients needing treatment are started on tablets but in some patients more complicated treatments are started due to the nature and severity of their condition. More details about the treatment options available can be found on the treatments page of PH Association's website.

You may also be given information about clinical drug trials that you may be eligible to enrol in. You are under no pressure to participate and it will not affect your ongoing care if you decide you do not wish to participate. Unfortunately despite the increasing number of treatments available we are unable to cure pulmonary arterial hypertension and therefore we liaise closely with the transplant team at Papworth.

Follow up

After we have confirmed a diagnosis of pulmonary hypertension patients are usually reviewed on a regular basis (approximately every 3-6 months). We have developed outreach clinics in Coventry (Coventry University Hospitals Coventry & Warwickshire), Norwich (Norfolk and Norwich Hospital) and Plymouth (Derriford Hospital) to make it easier for our patients who live some distance from Royal Papworth Hospital. We also recommend that patients remain in close contact with their local medical team to ensure that day to day issues can be managed. We work closely with your GP and local hospital team to ensure you receive the best possible care.

Papworth PH support group: PH matters

PH Matters is a patient led support group with active involvement of Royal Papworth PVDU team. The group meets regularly on a bimonthly basis and all newly diagnosed patients are provided with contact details for the group. A regular newsletter is issued via email to all members. The group is open to anyone affected by PH. You can contact the PH Matters support group by email using royalpapworthphmatters@gmail.com
You do not need to be a patient at Royal Papworth Hospital. It is supported by Papworth Hospital and the PH Association (PHA). For further information please ask at the hospital or contact PALS.

Useful links

What Is Pulmonary Hypertension - (External website)

Patient guides

Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Chronic Thromboembolic Pulmonary Hypertension FAQs