Pulmonary hypertension service for healthcare professionals
- What is pulmonary hypertension
- Current estimated incidence and prevalence
- Who to refer
- Follow-up and management of patients
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.
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.
Associated Risk Factors
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
- 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 CDROM.
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.
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.
- Pulmonary hypertension service
- The staff
- Information for patients
- For healthcare professionals
- Pulmonary endarterectomy