CTEPH is a form of pulmonary hypertension (PH). It develops when thromboembolic material obstructs pulmonary artery branches and mean pulmonary arterial pressure (mPAP) rises to ≥25 mmHg.1 The European Society of Cardiology (ESC) describes it as follows:1 “(in CTEPH) pathological lesions are characterized by organized thrombi tightly attached to the pulmonary arterial medial layer in the elastic pulmonary arteries, replacing the normal intima”
CTEPH is quite rare. Although its exact prevalence is unknown, it has been estimated at between 8 and 40 cases per million.2
In patients with mPAP of >50mmHg, 3-year mortality is up to 90%.3 Right ventricular heart failure is the most common cause of death. However, CTEPH is potentially curable – provided patients are eligible, the surgical procedure known as pulmonary endarterectomy (PEA) can prove effective.4 The level of activity, quality of life and life expectancy are significantly improved in a large proportion of patients who undergo this operation, meaning the procedure can effectively be thought of as a cure for many of them.5
It is essential that patients suspected of suffering from CTEPH (or any form of PH) be referred to a specialist centre. These centres are highly experienced in diagnosing the various types of PH. Diagnosis should be made with a matter of urgency, since the disease progresses quickly.
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