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)

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.

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

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.

Suggestive Symptoms		Associated Risk Factors
Fatigue Exertional dyspnoea Syncope/presyncope Peripheral oedema Atypical chest pain Palpitations +/- Raynaud’s phenomenon Systemic sclerosis/connective tissue disease		Congenital heart disease Family history of IPAH Recurrent pulmonary embolism HIV infection Advanced liver disease Known history of venous thromboembolism

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

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

Papworth Hospital NHS Foundation Trust
Papworth Everard
Cambridgeshire
CB23 8RE UK
Fax: 01480 364267
Phone: 01480 830541

A printable copy of these referral guidelines is available here.

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 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.