CTEPH (chronic thromboembolic pulmonary hypertension): symptoms, diagnosis and treatment

Pulmonary hypertension is, in the NHS description, high blood pressure in the blood vessels that supply the lungs, the pulmonary arteries. It is a serious condition that can damage the right side of the heart, because the heart has to work harder to push blood through narrowed vessels. If the right side of the heart continually works harder, it can gradually become weaker, which can lead to heart failure.

CTEPH is one of the types of pulmonary hypertension. The NHS says it is caused by scars from previous blood clots that narrow or block the pulmonary arteries, and that a blood clot blocking one of the vessels supplying your lungs is called a pulmonary embolism. In most people a clot in the lung is dissolved by the body and by blood-thinning treatment, but in some it leaves behind scarring that persists.

Pulmonary Hypertension Association UK describes pulmonary hypertension as an umbrella term for high blood pressure in the lungs from any cause, and lists CTEPH as group four, due to chronic blood clots in the arteries of the lung. Knowing the group matters because it points towards different treatments.

The NHS lists the symptoms of pulmonary hypertension as shortness of breath, tiredness, feeling faint or dizzy, chest pain, a racing heartbeat and swelling in the legs, ankles, feet or tummy. They often get worse during exercise, which can limit your ability to take part in physical activities.

Pulmonary Hypertension Association UK groups severity by how much exercise you can do before noticing symptoms such as breathlessness, from no symptoms with ordinary activity to symptoms at rest. This functional class helps the team judge how much pulmonary hypertension is affecting you and choose treatment.

The NHS notes that correctly diagnosing pulmonary hypertension can sometimes take time because its symptoms are similar to those of many other heart and lung conditions. Ongoing breathlessness after a known blood clot in the lung is a reason to tell your GP, so that the possibility can be considered rather than assumed to be deconditioning or asthma.

The NHS says to see a GP if you have any symptoms of pulmonary hypertension. It says to call 999 or go to A&E if you get sudden chest pain that does not stop after a few minutes, or heart palpitations with chest pain, shortness of breath or feeling faint, which could be a sign of another condition such as a blood clot in the lungs or a serious heart problem.

The NHS says tests for pulmonary hypertension include an echocardiogram, a type of heart scan, and right heart catheterisation, in which a thin, flexible tube is inserted into your pulmonary artery. Pulmonary Hypertension Association UK also lists chest X-ray, ECG, lung function tests, CT and high-resolution CT scanning, ventilation-perfusion scanning, pulmonary angiography, MRI and walk tests among the tests used for pulmonary hypertension.

To identify CTEPH specifically, specialist teams look for evidence of old clots in the pulmonary arteries using lung imaging such as a ventilation-perfusion scan or CT pulmonary angiography. The exact combination depends on the team and on your situation, and the sources used for this guide do not set out a single pathway.

Because treatment options differ by type, the NHS says that if pulmonary hypertension is caused by blood clots that block the pulmonary arteries, you may be offered anticoagulant medicines to prevent more clots forming and may also be offered an operation known as a pulmonary endarterectomy.

The NHS says pulmonary hypertension cannot be cured but treatments can reduce symptoms and help you manage the condition, and that if the cause is identified and treated early it may be possible to prevent permanent damage to the pulmonary arteries.

The NHS says pulmonary hypertension usually gets worse over time, and that left untreated it may cause heart failure, which can be fatal, so it is important to start treatment as soon as possible. It says the outlook varies depending on what is causing it, how quickly it is diagnosed, how advanced the symptoms are and whether there is another underlying health condition. The specialist in charge of your care can give more detailed information about your own situation.

Because CTEPH may be treatable by an operation or a procedure in a way that other forms are not, an early referral to a specialist centre is particularly worthwhile. That is the main reason ongoing unexplained breathlessness after a lung clot should be raised with a doctor.

The NHS says having pulmonary hypertension can affect your ability to carry out everyday activities. Pulmonary Hypertension Association UK is a charity that offers practical information and support for people living with the condition and their families, including information on exercise, driving, travelling, emotional wellbeing and benefits.

Regular review at the specialist centre, taking anticoagulant medicines exactly as prescribed and telling the team about any new symptoms are the practical foundations of day-to-day care. Ask the team about pregnancy, travel and exercise before making plans, because advice differs between people.

On the NHS, care for pulmonary hypertension is concentrated in designated specialist centres, which the NHS says number 7 in England and 1 in Scotland, with shared care arrangements for follow-up closer to home. The surgery for CTEPH is highly specialised and is done in a small number of centres. Private care exists for assessment and some treatment, but the same expertise and the same teams are involved in many cases, so it is worth asking any provider how many people with CTEPH they treat each year and how they link with the national specialist centres.