The main treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) is radical surgery, pulmonary thromboendarterectomy (PEA). However, about 40% of patients with CTEPH are inoperable due to distal pulmonary vascular lesions or the severity of hemodynamic disorders. Almost 30% of patients with CTEPH experience persistent or recurrent pulmonary hypertension after surgery, that requires a drug treatment with PAH-specific drugs. This review presents current data on the use of targeted therapy in patients with CTEPH. The review addresses the place, indications, and the evidence base for using the main groups of specific drugs, including stimulators of soluble guanylate cyclase, phosphodiesterase type 5 inhibitors, endothelin receptor antagonists, and prostacyclin analogues.

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http://dx.doi.org/10.18087/cardio.2024.12.n2784DOI Listing

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