Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disease with significant morbidity. It is a result of an alternate natural history in which there is limited resolution of thromboemboli with pulmonary artery obstruction leading to pulmonary hypertension (PH). CTEPH requires a thorough clinical assessment including pulmonary hemodynamics and radiologic evaluation in addition to consultation with an expert center. Surgical intervention remains the optimal management strategy. Select patients may be candidates for catheter-based intervention with balloon pulmonary angioplasty in centers with clinical expertise. Inoperable patients or those with post-intervention PH are treated with pulmonary hypertension-targeted medical therapy.
Keywords: Balloon pulmonary angioplasty (BPA); Chronic thromboembolic disease (CTED); Chronic thromboembolic pulmonary hypertension (CTEPH); Pulmonary endarterectomy (PEA); Pulmonary hypertension (PH); Pulmonary thromboendarterectomy (PTE).
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