This review presents an analysis of recent publications on the principles of diagnosis and management of patients with chronic thromboembolic pulmonary hypertension (CTEPH). The authors emphasize the importance of patient's operability assessment regarding the possibility of performing pulmonary thrombendarterectomy - a radical treatment option due to complete post-thrombotic material removal from the branches of the pulmonary artery. In the case of an inoperable type of CTEPH, such a method of transcatheter treatment as step-by-step balloon angioplasty of the pulmonary artery (BPA) is proposed. The article clearly outlines the principles of selection, patient training and techniques for BPA. The authors demonstrated their own patented method of the peripheral vascular bed visualization in the case of recanalization of chronic occlusions, which increases the safety of this intervention. Particular attention is paid to hybrid approaches using a soluble guanylate cyclase stimulator, riociguate in combination with BPA for high risk CTEPH patients. The review presents the data of the post hoc analysis of the CHEST-1 study and the data of real clinical practice of the use of riociguat in combination with the endovascular method of treatment on the example of the leading expert groups in Europe, who demonstrated in their studies the positive effect of riociguat on the hemodynamic parameters and the frequency of perioperative complications.