[Chronic thromboembolic pulmonary hypertension: from pathogenesis to the choice of treatment tactics].

Presents data on the pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH), which serve as a rationale for approaches to the choice of treatment. CTEPH usually begins with persistent obstruction of the large and/or medium pulmonary arteries by organized thrombi. Impaired lysis of thrombi may be associated with abnormal fibrinolysis, hematological or autoimmune diseases.

[Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry].

Aim: To assess demographic and clinical characteristics, to describe of the functional and hemodynamic status, profile of concomitant pathology, data of instrumental examination in chronic thromboembolic pulmonary hypertension (CTEPH) patients; to study the features of specific and supportive therapy according to the data of the Russian national registry.

Materials And Methods: From 2012 till 2020 a multicenter, prospective study in 15 regional expert centers of the Russian Federation (www.medibase.

[A rare clinical case of the patient with pulmonary hypertension associated with hereditary hemorrhagic telangiectasia. Case report].

The article describes a rare clinical case of the patient with pulmonary hypertension associated with hereditary hemorrhagic telangiectasia and discusses the issues of the treatment choice of these patients. The presented clinical case is of interest because pulmonary hypertension was initially revealed, then its hereditary nature was confirmed and OslerWeberRendu disease was diagnosed.

[Influence of demographic characteristics on the clinical course and results of therapy in patients with precapillary pulmonary hypertension].

Aim: To assess the clinical course of the disease and the features of the treatment goals achievement in patients with IPAH and inoperable CTEPH depending on gender and age at the time of diagnosis verification.

Materials And Methods: The study included 88 patients with IPAH and 38 patients with inoperable CTEPH with a PAWP 12 mm Hg and the duration of PAH-specific therapy treatment more than 12 months. IPAH/CTEPH patients were divided into groups depending on age at the time of diagnosis verification: age 50 years (n=69)/ 50 years (n=57), and gender: 106 women/20 men.

[The achievements of the modern specific therapy of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: focus on the stimulator of soluble guanylate cyclase riociguat].

Currently, treatment of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) is focused on three signaling pathways: the NO pathway, the endothelin pathway, and the prostacyclin pathway. Riociguat is the only representative of stimulators of the soluble guanylate cyclase (sGC) class that is approved for the treatment of PAH and inoperable and persistent/recurrent CTEPH. The review presents data from clinical trials showing a positive effect of riociguat on the functional and hemodynamic profile of patients with PAH and CTEPH.