Chronic Thromboembolic Pulmonary Hypertension

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  • Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disease with significant morbidity. It is a long-term complication of pulmonary embolism (PE), but also occurs in patients with no history of PE, including those without a spleen, with some blood clotting disorders and in cases of pacemaker infection.

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    Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disease with significant morbidity. It is a long-term complication of pulmonary embolism (PE), but also occurs in patients with no history of PE, including those without a spleen, with some blood clotting disorders and in cases of pacemaker infection.

    Once the diagnosis is confirmed, the treatment of choice is pulmonary endarterectomy to remove the clots. This effectively lowers the pulmonary vascular resistance and normalises resting pulmonary artery pressures, leading to recovery of the right ventricle. When pulmonary endarterectomy is not feasible, percutaneous balloon pulmonary angioplasty is alternative, but repeat procedures are generally required. Pharmaceutical therapy may help to improve symptoms and haemodynamics. Current studies are focusing on strategies for earlier CTEPH diagnosis after acute PE, as well as the optimal treatment of inoperable patients.

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