What is pulmonary hypertension?

Pulmonary hypertension describes a diverse group of diseases which result in mean pulmonary artery pressure ≥ 25mmHg at rest. The underlying pathophysiology is that of progressive pulmonary vascular remodelling and subsequent right ventricular dysfunction and failure.

Causes of pulmonary hypertension

Group 1: Pulmonary arterial hypertension

  • Idiopathic
  • Hereditable
  • Associated with: connective tissue disease, congenital heart disease, portal hypertension, HIV infection, drugs and toxins
  • Pulmonary veno-occlusive disease
  • Pulmonary haemangiomatosis

Group 2: PH due to left heart disease
Group 3: PH due to lung diseases/hypoxia
Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH)
Group 5: Miscellaneous

  • Sarcoid
  • Histiocytosis X
  • Lymphangiomatosis

Current estimated incidence and prevalence

While idiopathic pulmonary arterial hypertension (formerly primary pulmonary hypertension) remains an uncommon condition, pulmonary hypertension associated with other conditions is not that rare. The recognition of pulmonary arterial hypertension is increasing (incidence currently estimated at 8 cases/ million/year, prevalence 20-50 cases /million). The availability of effective therapies and the increased recognition of pulmonary arterial hypertension in association with other conditions has resulted in the development of nationally designated specialist centres such as the Pulmonary Vascular Disease Unit (PVDU) at Royal Papworth Hospital.

The incidence of chronic thromboembolic pulmonary hypertension is also greater than initially suggested (up to 4% of patients with pulmonary embolism after two years). Royal Papworth Hospital has also been commissioned as the national referral centre for pulmonary endarterectomy.


Suggestive symptoms Associated risk factors
Extertional dyspnoea Congenital heart disease
Syncope/presyncope Family history of IPAH
Peripheral oedema Recurrent pulmonary embolism
Atypical chest pain HIV infection
Palpitations Advanced liver disease
+/- Reynaud's phenomenon Known history of venous thromboembolism
Systemic sclerosis/connective tissue disease  


Who to refer

The PVDU encourages you to promptly refer any patient aged over 16 who you believe has:

  • Unexplained pulmonary hypertension
  • Pulmonary arterial hypertension (see causes above)
  • Chronic thromboembolic pulmonary hypertension
  • Miscellaneous causes of pulmonary hypertension
  • Patients with PH associated to left heart disease and/or lung disease are reviewed on an individual basis.

Echocardiography should be used as the primary screening modality, with pulmonary hypertension being suggested by:

  • Estimated pulmonary artery systolic pressure of ≥ 40mmHg + RA pressure (Tricuspid regurgitant jet velocity of 2-8-3.4m/s)
  • Evidence of significant right ventricular dilation or dysfunction
  • Absence of left ventricular dysfunction

For patients with systemic sclerosis we recommend using the DETECT screening tool. A link to the online calculator is here, but Android and Apple apps are available for your phone. This is based on a multicentre multinational screening study.


Suggested investigations prior to referral:

  • Chest radiograph
  • Electrocardiograph
  • Full lung function tests
  • Echocardiography
  • Pulse oximetry at rest +/- arterial blood gases
  • Renal and liver function tests
  • Autoantibody screen
  • Consider ventilation/perfusion scan or CT pulmonary angiography

Please include copies of any radiological investigations and reports. Where possible we would prefer advanced imaging to be sent either via an electronic image link or on CD.


How to refer a new patient

Non urgent

Please email papworth.phreferrals@nhs.net

Urgent referral/case discussion
Pulmonary hypertension consultant on-call: via hospital switchboard

Royal Papworth Hospital NHS Foundation Trust
Papworth Road
Cambridgeshire Biomedical Campus
Phone: 01223 638000

A printable copy of these referral guidelines is available online.


Management and follow-up of pulmonary hypertension patients

Typically we follow-up patients every 3-6 months. Since many patients are referred from a long distance, we ask that they remain under the joint care of the GP, local hospital consultant and the local pulmonary hypertension service. We are seeking to develop shared care of patients requiring long-term follow-up with interested physicians (in place with Coventry University Hospital, at University Hospitals Coventry & Warwickshire, Derriford Hospital at Plymouth and Norfolk and Norwich Hospital at Norwich). Please contact us for more details. We will be happy to discuss individual patient follow-up with you.


Pulmonary arterial hypertension targeted therapies used:

Over the past 10 years there have been a number of treatments licensed for pulmonary arterial hypertension. These drugs are backed up with clinical trial evidence to support their use. The Royal Papworth PVDU is responsible for the prescription and delivery of the pulmonary arterial hypertension targeted drugs but the general practioner remains responsible for the prescription of any other drugs the patient is taking. Patients and healthcare professionals are to contact us for advice 24 hours a day, 365 days a year.

Phosphodiesterase type 5 inhibitors (Sildenafil, Tadalafil)

Usually first line treatment. Sildenafil is taken three times per day and tadalafil once daily. Common side effects include flushing, headache, systemic hypotension, nasal stuffiness, epistaxis and gastro-oesophageal reflux. It can rarely cause non-arteritic anterior ischaemic optic neuropathy (need to discontinue if sudden visual disturbance occurs).

Endothelin receptor antagonists (Bosentan, Ambrisentan and Macitentan)

Bosentan is taken twice daily whereas Ambrisentan and Macitentan are taken once daily. Common side effects include: flushing, headache, systemic hypotension, ankle oedema, anaemia and abnormal LFTs (ALT, AST). In view of the side effect patients are required to have their haemoglobin checked monthly for the first month and then three-monthly and LFTs checked monthly.

Prostenoids (nebulised Iloprost, intravenous Epoprostenol)

Prostenoid treaments used in patients with more advanced pulmonary hypertension and require educating the patient and relatives about how to make up and use the treatments.

Nebulised Iloprost is given through a special nebuliser rather than the standard nebuliser used for bronchodilators. The drug has a short half-life and therefore needs to be inhaled every three hours (seven times per day). Common side effects include: flushing, headache, jaw ache, cough, wheezing, systemic hypotension and diarrhoea.

Continuous intravenous Epoprostenol is given via a Hickman line using a pump. The drug has a very short half-life (3-5 minutes) and therefore cannot be stopped abruptly. Patients and relatives are given extensive training and support when initiated on Epoprostenol. Prior to discharge they are assessed to ensure they are competent to manage the infusion and what to if they have any problems. Common side effects include: headache, flushing, jaw ache, diarrhoea, nausea and sepsis related to Hickman line.

Pulmonary hypertension published guidelines

ESC/ERS 2015 Pulmonary Hypertension guidelines