[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.

[The soluble guanylate cyclase stimulator riociguat is the first-line therapy for chronic thromboembolic pulmonary hypertension patients].

Chronic thromboembolic pulmonary hypertension (CTEPH) is a precapillary type of pulmonary hypertension with chronic obstruction of large and medium branches of pulmonary arteries along with secondary alterations in pulmonary microcirculation, which cause progressive increases in pulmonary vascular resistance and pulmonary arterial pressure and ensuing severe right heart dysfunction and heart failure. Pulmonary thromboendarterectomy (PTE) is the treatment of choice for CTEPH; however, this procedure is available not for all patients. Although the surgery performed in the conditions of centers with advanced experience generally shows good results, up to 40% of patients are technically inoperable or PTE is associated with a high risk of complications.

Clinical and Instrumental Characteristics of Newly Diagnosed Patients with Various Forms of Pulmonary Hypertension according to the Russian National Registry.

Aim: To study demographic and clinical characteristics and to give a comparative description of the functional and hemodynamic status, profile of concomitant pathology in patients with various forms of pulmonary arterial hypertension (PAH), and chronic thromboembolic pulmonary hypertension (CTEPH) according to the Russian National Registry.

Methods: During the period from January 01, 2012, till January 01, 2019, 1105 patients aged >18 years with verified diagnosis of PAH and CTEPH, who were subsequently observed at 15 PH expert centers of the Russian Federation in the 52 provinces, are included in the Russian registry on the basis of the Federal State Budgetary Institution of Cardiology of the Ministry of Healthcare of Russia. All newly diagnosed patients ( = 727) were entered into the registry database (NCT03707561).

[Features of clinical, functional and hemodynamics profile, medical treatment and prognosis evaluation in patients with inoperable chronic thromboembolic pulmonary hypertension and idiopathic pulmonary arterial hypertension according to the Russian registry].

Aim: to perform the complex analysis of clinical, functional, hemodynamics profile in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) compared to the idiopathic pulmonary arterial hypertension (IPAH) group at the time of diagnosis verification according to the Russian registry, and to evaluate the features of medical therapy and it's influence on prognosis in these patients.

Materials And Methods: In the retrospective study 193 patients with IPAH and 130 patients with inoperable CTEPH older than 18 years were included. All included patients were the participants of Russian registry (www.

Modern view on the place of riociguat in the treatment of pulmonary hypertension.

Current research is aimed at studying the fundamental therapeutic targets and discovering new drugs acting on previously set targets. Until recently, the only therapeutic strategy to influence the molecular pathway of nitric oxide (NO) - soluble guanylate cyclase (sGC) - cyclic guanosine monophosphate (cGMP) was the use of phosphodiesterase type 5 inhibitors (PDE-5 inhibitors), such as sildenafil. In September 2014, the first member of sGC stimulators riociguat was licensed in Russia.

The economic burden of chronic thromboembolic pulmonary hypertension in Russian Federation.

Aim: The aim of current study was to estimate the economic burden of the chronic thromboembolic pulmonary hypertension (CTEPH) in Russia based on patient registry.

Materials And Methods: Cost of illness study was based on data derived from CTEPH patient registry that was developed at the Division of hypertension of FSBI "National Medical Research Center of Cardiology". Demographic and clinical patient characteristics were analyzed with descriptive statistic methods.

[Bosentan use in pulmonary arterial hypertension: Russian and foreign experience].

The results of evaluating the efficacy and safety of bosentan in patients with pulmonary arterial hypertension (PAH), as shown by the data of foreign randomized controlled trials and the authors' own experience, convincingly demonstrate that the introduction of the drug into clinical practice has led to a significant improvement of the possibilities of drug therapy in patients with this serious illness. Bosentan substantially improves physical activity in patients, reduces the severity of clinical symptoms, slows down the rates of disease progression, and prolongs survival in patients with different forms of PAH.

[Pulmonary hypertension in Russia: the first results of the national register].

Aim: To investigate the demographic and clinical characteristics of patients with pulmonary hypertension (PH) and chronic thromboembolic pulmonary hypertension (CTEPH), regions of their residence, the specific features of diagnosis and drug therapy, and the patients' survival within the framework of a multicenter open-label prospective study.

Subjects And Methods: The study enrolled patients over 18 years of age with PH classified as Group 1 (PH) and Group 4 (CTEPH). The follow-up was made in 9 expert centers of Russia on January 1 to February 28, 2014.

[24-hour systolic wave increment index monitoring in patients with low-renin arterial hypertension].

Aim: To analyze the circadian rhythm of blood pressure (BP) and daily reflected wave values in patients with low-renin hypertension with normal and elevated aldosterone production.

Subjects And Methods: The investigation included 66 patients. 24-hour BP monitoring was carried out and arterial wall rigidity and reflected wave values were assessed in all the patients.

Impact of echinococcosis on quality of sheep meat in the South eastern Kazakhstan.

A survey of 2,123 slaughtered adult sheep in markets of the south-east Kazakhstan established that the average contamination of animals by Echinococcus granulosus is 9.1% with primary localization of cysts in livers and lungs. The histologic structure of the muscle tissue of infected sheep displayed destructive changes with a prevalence of granular dystrophy.