Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare cause of pulmonary hypertension; less than 5% of pulmonary hypertension is caused by recurrent pulmonary thromboembolism (PTE). By definition, CTEPH happens within the first two years after symptomatic PTE; however, cases are often diagnosed without a history of acute PTE. Because of the poor functional status and chronicity of this disease, the classic and curative strategy of open pulmonary endarterectomy cannot be applied in some patients with lesions that involve the distal subsegmental pulmonary artery. Bridging therapy is needed for cases that are technically operable but have an unacceptable risk-benefit assessment or residual symptomatic pulmonary hypertension following surgical removal. Groups in Europe and Japan recently introduced balloon pulmonary angioplasty or percutaneous transluminal pulmonary angioplasty, which has led to significant improvement in functional and hemodynamic parameters in patients with CTEPH. This article introduces recent updates in patient selection and interventional procedures for this chronic and devastating disease.

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