Pulmonary hypertension

The Royal Free London is a national centre for diagnosis and treatment of pulmonary arterial hypertension (PAH). We are integrally linked with the connective tissue disease service at the Royal Free Hospital and enjoy close links with the interstitial lung disease service at the Royal Brompton Hospital. We have wide experience in all types of pulmonary hypertension and provide support to other centres in the UK.

What is pulmonary hypertension?

Watch our short film which explains what pulmonary hypertension is (duration 4:40).

Pulmonary hypertension is defined by a high pressure in the pulmonary arteries (25 mmHg or above). The most common causes are left heart disease (systolic or diastolic dysfunction or severe valvular disease), hypoxic lung disease (such as interstitial lung disease or COPD) or pulmonary emboli.

Pulmonary arterial hypertension on the other hand is an uncommon group of diseases where the pulmonary hypertension is due to disease of the pulmonary arterioles. The principal causes of pulmonary arterial hypertension are:

• Idiopathic – often found in young women.
• Connective tissue disease – most commonly scleroderma and scleroderma overlap.
• Congential heart disease – in association with Eisenmenger’s syndrome.
• Rare causes – there are many, including cirrhosis with portal hypertension, HIV, Schistosomiasis, sickle cell disease and use of slimming tablets like fenfluramine.

Although this distinction between pulmonary arterial hypertension (PAH) and pulmonary hypertension (PH) is pivotal in deciding treatment options, the terms are often used interchangeably particularly when talking to patients.

Pulmonary hypertension is a sequel of pulmonary emboli in 2-4% of cases. This can sometimes be treated with surgical removal of the occlusive material. Although not strictly part of PAH, the investigations and management overlap so these patients are also managed by pulmonary hypertension specialists.

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Referral details

At the Royal Free London we are pleased to see any patient suspected of having pulmonary hypertension.

Patients who are breathless usually undergo investigations including an ECG, echocardiography, pulmonary function tests, chest x-ray and if necessary a CT chest scan before being referred for consideration of pulmonary hypertension.

Pulmonary hypertension should be considered in patients with unexplained progressive breathlessness, unexplained right heart failure or exertional presyncope or syncope. It should be considered particularly in patients with conditions that can predispose to pulmonary hypertension such as congenital heart disease, connective tissue disease, liver failure, pulmonary emboli and HIV.

If you are a GP or other health professional and would like to refer us a patient, please send a letter together with these investigation results to us. In urgent cases, please contact the pulmonary hypertension fellow or cardiology registrar on call to discuss the case with us.

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