Information for healthcare professionals
- Pulmonary vascular diseases unit
- What is pulmonary hypertension
- Current estimated incidence and prevalence
- Who to refer
- Follow-up and management of patients
The pulmonary vascular diseases unit (PVDU) at Papworth Hospital is an internationally recognised centre for the management of pulmonary hypertension and other pulmonary vascular disorders. We are part of the UK National Pulmonary Hypertension Service and the nationally designated centre performing pulmonary endarterectomy surgery for chronic thromboembolic hypertension. We provide a patient centred and multidisciplinary team approach to the evaluation, investigation and management of patients with 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
- 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
- Histiocytosis X
Whilst 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 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). Papworth Hospital has also been commissioned as the national referral centre for pulmonary endarterectomy.
Associated Risk Factors
Who to refer
The PVDU encourages you to promptly refer any patient aged older than 16 whom you believe has:
- Unexplained pulmonary hypertension
- Pulmonary arterial hypertension (see causes above)
- Chronic thromboembolic pulmonary hypertension
- Miscellaneous causes of pulmonary hypertension
We do not accept referrals for patients with PH associated with left heart disease.
Echocardiography should be used as the primary screening modality, with pulmonary hypertension being suggested by:
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
- Full lung function tests
- 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.
Urgent referral/case discussion
Pulmonary hypertension consultant on-call - via hospital switchboard
Papworth Hospital NHS Foundation Trust
CB23 8RE UK
Fax: 01480 364267
Phone: 01480 830541
A printable copy of these referral guidelines is available here.
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 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 last 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 in pulmonary arterial hypertension. The 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 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
- Pulmonary hypertension service
- Contacts and useful links
- The staff
- Information for patients
- For healthcare professionals
- Pulmonary endarterectomy
- Current Projects
Assessing how the heart adapts to pulmonary hypertension