Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) remains a rare and underdiagnosed disease. After one or several episodes of acute pulmonary embolism, around 3% of patients develop CTEPH and two-thirds of these patients are potential surgical candidates. Besides surgery, additional treatment modalities are pulmonary arterial hypertension medication and balloon pulmonary angioplasty. Patients should be evaluated in CTEPH expert centers to ensure the most appropriate therapy. Pulmonary endarterectomy (PEA) is a complex, but standardized surgical procedure aiming to clear the obstructed pulmonary arteries completely. For optimal visualization, deep hypothermic circulatory arrest is a prerequisite. This article will give an overview of the evaluation, indication and surgical management of patients with CTEPH.

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