Pulmonary hypertension


Pulmonary hypertension is high blood pressure in the blood vessels that supply the lungs (pulmonary arteries).  It is a serious condition that can damage the right side of the heart.  The walls of the pulmonary arteries become thick and stiff, and cannot expand as well to allow blood through.  The reduced blood flow makes it harder for the right-hand side of the heart to pump blood through the arteries.  If the right-hand side of your heart has to continually work harder, it can gradually become weaker, which can lead to heart failure.  Pulmonary hypertension is a rare condition that can affect people of all ages, but it is more common in people who have another heart or lung condition


Signs and symptoms

The symptoms of pulmonary hypertension include the following:

  • Shortness of breath
  • Fatigue
  • Chest pain
  • Palpitations (heartbeat rate increased)
  • Pain (right side of the abdomen)
  • Poor appetite
  • Lightheadedness
  • Fainting or syncope
  • Swelling (legs/ankles)
  • Cyanosis


Less common signs/symptoms include non-productive cough and exercise-induced nausea and vomiting


Causes of pulmonary hypertension

The changes in the pulmonary arteries that lead to pulmonary hypertension can be caused by:

  • problems with the smaller branches of the pulmonary arteries (PAH)
  • conditions that affect the left side of the heart
  • lung diseases or a shortage of oxygen in the body (hypoxia)
  • blood clots that cause narrowing or a blockage in the pulmonary arteries


Initial assessment

NOTEPulmonary hypertension can be difficult to diagnose because the symptoms are similar to those of other heart or lungs conditions

Ask about:

  • symptoms and how they affect the person's life
  • family history – although rare, pulmonary arterial hypertension can run in families
  • any current medication - a history of exposure to drugs such as benfluorex (a fenfluramine derivative), dasatinib, cocaine, methamphetamine, ethanol leading to cirrhosis, and tobacco leading to emphysema is considered significant
  • any other medical conditions


A physical examination should be performed to look for typical signs of pulmonary hypertension (see above for signs/symptoms), including listening to the person's heart and lungs, and checking for any swelling of the legs and ankles



If pulmonary hypertension is suspected based on the above assessments, echocardiography should be performed as the next step


Other tests that may be considered include:

  1. an ECG
  2. chest X-ray – to check for symptoms such as an enlarged heart or scarring in the lungs, which can cause shortness of breath
  3. lung function tests
  4. exercise tests 
  5. a ventilation-perfusion scan – to look for blood clots that may be causing pulmonary hypertension
  6. blood tests – to rule out other possible conditions, such as thyroid and liver disease


Referral and treatment

If a person is suspected as having pulmonary hypertension they should be admitted to hospital

Pulmonary hypertension cannot be cured, but treatments to reduce symptoms and manage the condition are available.  If the cause is identified and treated early, it may be possible to prevent permanent damage to the pulmonary arteries

If pulmonary hypertension is caused by another condition, such as a heart or lung problem, treatments will focus on the underlying condition

If pulmonary hypertension is caused by blood clots that block the pulmonary arteries, you may be offered anticoagulant medicines to prevent more clots forming.  Patients with blood clots may also need to have a procedure such as pulmonary endarterectomy or balloon pulmonary angioplasty, depending on their clinical circumstances



An electrocardiogram (ECG) can usually be undertaken in primary care.  If your practice does not offer ECGs, please request an ECG from East Kent Hospitals


Chest radiography

For chest x-rays, you can refer the patient to the Radiology Department at East Kent Hospitals (find details here)


Blood tests / Phlebotomy

If your practice does not have a practice nurse who is trained to take bloods, you can refer a patient to the Pathology Department at East Kent Hospitals for a blood test (find details here)

Alternatively, Buckland Hospital (Dover) and the Royal Victoria Hospital (Folkestone) both operate a walk-in service where no appointment is necessary, except for if the patient requires a Glucose Tolerance Test (GTT).  In the event that a GTT is required, please call 01304 222552 (for Buckland) and 01303 854484 (for Royal Victoria) to arrange a suitable appointment

Please ensure that the patient remembers to take with them their blood test form to the walk-in centres

Advice and Guidance is being made available for all specialties, and is being provided by consultant specialists at East Kent Hospitals.  To make a request or to check to if a query has been answered, you will need to log in via the electronic Referral System (eRS)

Click here for the "how to access" e-Referral Advice and Guidance Manual for instructions on how to make a request and check responses

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