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Pulmonary hypertension service for healthcare professionals

What is pulmonary hypertension?

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

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 NCG 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
  • +/- 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 established pulmonary hypertension.

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

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 CDROM.

How to refer a new patient

Non urgent
Dr Joanna Pepke-Zaba or Dr Karen Sheares - Consultant Respiratory Physicians

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

Papworth Hospital NHS Foundation Trust
Papworth Everard
Fax: 01480 364267
Phone: 01480 830541

A printable copy of these referral guidelines is available here.

Management and follow-up of pulmonary hypertension patients

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). Please contact us for more details. We will be happy to discuss individual patient follow-up with you.

See also:


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