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Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique pulmonary vascular disease caused by chronic block of the major lung arteries. Signs and symptoms commonly include progressive breathing difficulties (dyspnea) on exertion, fatigue, palpitations, loss of consciousness (syncope), or swelling (edema). The disease may appear a few months or many years after the sudden blockage in a lung artery by a blood clot (acute pulmonary embolism). However, up to 60% of patients have no history of acute pulmonary embolism. Some people with this disease may have clotting problems. Research suggests there may be a genetic predisposition leading to abnormal vascular healing after pulmonary embolism in susceptible individuals, but no specific gene mutations have been identified in CTEPH. Curative treatment is done with pulmonary endarterectomy (a surgery that removes the blood clots in the lungs), and more recently by pulmonary balloon angioplasty (a procedure that opens up the vessels of the lung and increases blood flow).
Source: GARD Last updated on 05-01-20
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