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Aim: To analyze the relationship between metabolic disorders and obesity with hemodynamic parameters and the severity of the condition of patients with chronic thromboembolic pulmonary hypertension (CTEPH) based on the POLET register., Materials and Methods: The study included patients with CTEPH from the POLET register. Data from medical history, lipid profile, glycemia, uric acid level, weight, height, functional class (FC), distance in the 6-minute walk test (6MWT), echocardiography, and right heart catheterization were used for analysis., Results: The study included 84 patients 60±14 years old, including 45 men. The majority of patients had FC III - 51 (65%), which corresponded to 6MWT - 338±113 m. Nineteen (23%) people had experience in taking PAH specific therapy, 37 (46%) had cardiovascular comorbidity. In the general group, the body mass index (BMI) was 25.7±4.5 kg/m 2 , and normal glycemia, lipid profile, and uric acid levels were also determined. mPAP was 51±12 mmHg, RA area was 25.5 (20; 30) cm 2 . Correlations were identified between: RA area and the level of total cholesterol (r=0.60; p <0.01), triglycerides (r=-0.24; p <0.001), LDL cholesterol (r=0.75; p = 0.04), uric acid (r=0.75; p =0.03); total cholesterol and 6MWT (r=0.44; p =0.04). Analysis of metabolic disorders, FC and comorbidity revealed differences between FC III and IV groups in the level of HDL cholesterol ( p =0.02) and triglycerides ( p <0.01)., Conclusion: The POLET registry includes mainly older patients. Severe FC (87% - III and IV) may be a result of the older age and the presence of concomitant pathology in almost half of the patients. The discovered relationships between the area of RA and FC with lipid and purine metabolism were discovered for the first time, however, they correspond to the direction of the world scientific researches, are important due to the use of these indicators in the scale for calculating the risk of death in patients with PAH and require further development.

The article reviews current approaches to diagnosing and treating chronic thromboembolic pulmonary hypertension (CTEPH). The definition of the CTEPH is given, and its main risk factors are described. It is shown that the modern algorithm of diagnostic search includes four stages; the examination methods used at each stage are characterized. The most rational approach to CTEPH therapy is provided; the possibilities and limitations of pulmonary endarterectomy, transluminal balloon angioplasty of the pulmonary arteries, and specific therapy of pulmonary hypertension in such patients are described. The clinical presentation and treatment of CTEPH according to the Russian Pulmonary Hypertension Registry are reviewed.

On December 13, 2021, an expert council was held to determine the position of experts of different specialties regarding the reasons for the low level of diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) in real clinical practice in a pandemic of a new coronavirus infection and possible ways to improve detection in patients with pulmonary embolism (PE) ) in history. The reasons for the low level of diagnosis of CTEPH are the insufficient level of knowledge of specialists, especially primary care physicians; lack of clear regulatory documents and expert centers for the management of this category of patients. Primary diagnosis of CTEPH in a pandemic can be strengthened through the widespread use of telemedicine for consultations of primary care physicians with specialists from expert centers; to maximize the role of echocardiography and computed tomography (CT) as differential diagnostic tools for dyspnea, in particular in patients with COVID-19. To increase the detection rate of CTEPH, diagnostic vigilance is required in patients with risk factors and episodes of venous thromboembolism. To improve the screening of CTEPH, it is necessary to create an algorithm for monitoring patients who have had PE; provide educational activities, including through the media; create materials for patients with accessible information. The regulatory documents should designate the circle of responsible specialists who will be engaged in long-term monitoring of patients with PE. Educational programs are needed for primary care physicians, cardiologists, and other physicians who come into the field of view of patients with CTEPH; introduction of a program to create expert centers for monitoring and managing patients with the possibility of performing ventilation-perfusion lung scintigraphy, cardiopulmonary stress test, CT, right heart catheterization. It seems important to build cooperation with the Ministry of Health of Russia in order to create special protocols, procedures for managing patients with PE and CTEPH.

For patients with pulmonary hypertension (PH) there have been no recommendations for physical rehabilitation for a long time, and in fact physical activity was discouraged. Nowadays, the position regarding exercise in patients with PH has changed studies have shown the safety and effectiveness of exercise in these patients. Most of the studies published to date on rehabilitation in patients with PH have assessed its effect on exercise tolerance. This review provides an overview of published studies and their conclusions on the effectiveness of rehabilitation in patients with chronic thromboembolic hypertension being as a complex therapy using medical and surgical methods of treatment and rehabilitation. The inclusion of rehabilitation in complex treatment allows to achieve patients highest possible tolerance to physical activity, increase in the functional class, quality of life and restore working capacity.

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.pro) included 404 newly diagnosed CTEPH patients over the age of 18 years in the Russian registry of patients with pulmonary arterial hypertension and CTEPH (NCT03707561). The diagnosis was established by European and Russian clinical guidelines for the diagnosis and management of pulmonary hypertension. 154 inoperable CTEPH patients an additional analysis of specific and supportive therapy was performed., Results: The study included 404 patients (55.6% women and 44.3% men) at the age of 58.6 [48.6; 69.3] years. Median time from symptom onset to the diagnosis verification waswas 2.4 years (from 0.1 to 2.9 years). 79.1% of patients were in the III and IV functional class (World Health Organization) at the time of diagnosis and in 44.1% with RHF (right heart failure). In assessing the profile of concomitant pathology, it was noted that CTEPH patients were more often with arterial hypertension (39.1%), erosive-ulcerative lesions of the stomach/duodenum (16.1%), atrial fibrillation (13.8%), obesity (13.1%). Distance in 6MWD (6-min walk distance) was 337.2 [250; 422] m, Borg dyspnea index scale 4.1 [3.0; 5.0] points. Hemodynamic parameters according to right heart catheterization were: Mean PAP (pulmonary arterial pressure) (51.114.04) mmHg, CO (cardiac output) (3.50.98) l/min, CI (cardiac index) (2.00.48) l/min/m2, PVR (2008528) dyns/cm5., Conclusion: According to the Russian registry, inoperable CTEPH patients had precapillary PH (pulmonary hypertension) with severe functional status, in combination with frequent concomitant pathology (arterial hypertension, erosive-ulcerative lesions of the stomach/duodenum, atrial fibrillation, obesity, right heart failure). 66% of inoperable CTEPH patients received specific drug therapy.

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.medibase.pro) in 15 Russian expert centers during the period from 01.01.2012 to 31.12.2018 year. The diagnosis was verified according to the algorithm, reflected in the Russian guidelines on diagnosis and treatment of PH and CTEPH (2016 year). The comparison analysis of clinical, functional, hemodynamics parameters in patients with IPAH and inoperable CTEPH was made., Results: The status of 193 patients with IPAH (32 male and 162 female) and 130 patients with inoperable CTEPH - (40 male and 90 female) was analyzed during the study. The CTEPH patients were older compared to the IPAH patients: 52.2 [41.1; 60.6] and 36.5 [26.8; 36.5] years, respectively. The median period since symptom occurrence till CTEPH verification was 1.08 [0.2; 3.1] years, in IPAH patients - 2.01 [0.6; 4.2] years. More than 80% of inoperable CTEPH patients had III and IV functional class (FC) according to the World Health Organization classification at the time of diagnosis verification versus 61% of IPAH patients. According to echocardiography the level of mean pulmonary arterial pressure was comparable in two groups of PH patients. However in inoperable CTEPH patients the right atrial area was larger. The significantly higher value of mean pulmonary arterial pressure and lower value oxygen saturation of arterial blood according to the right heart catheterization were revealed. The 5-year survival in CTEPH patients, receiving initial dual combination therapy in 75% cases (in 40% - combination of riociguat and iloprost ) was 93% versus 86.5% in patients with IPAH., Conclusion: It was revealed, that inoperable CTEPH patients were significantly older with severe functional and hemodynamics status at the time of diagnosis verification, although with higher level of 5-year survival compared to the IPAH patients according to the Russian registry.

Aim: To evaluate the effectiveness of balloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH)., Materials and Methods: Forty patients with inoperable CTEPH were enrolled in this study. The indications were determined by multidisciplinary team. The average age of patients was 53.5 [43; 63] years. In 65% of cases patients had functional class III (according to WHO); the distance in the 6-minute walk test (6MWD) was 327 [280; 400] m; catheterization of the right heart revealed systolic pulmonary artery pressure (SPAP) 82 [64; 100] mm Hg, mean pulmonary artery (mPAP) 48.5 [38; 56] mm Hg, pulmonary vascular resistance (PVR) 784 [525; 1257] dyn·s/cm-5. Each patient underwent 6 BPA., Results: The effectiveness of BPA was assessed 2 months after the last session. According to the data of right heart catheterization SPAP decreased by 27.3%, mPAP by 26%, PVR by 34.5% from baseline. After all series of BPA echocardiography and magnetic resonance imaging demonstrated reverse remodeling of the right heart. Also significant decrease in the level of BNP by 62%, increasing in 6MWD distance by 39% and improvement of the functional class up to I in 60% cases and up to II in 40% cases were noted., Conclusion: The results of the present study demonstrated a high efficacy of BPA allowing to normalize hemodynamic and clinical parameters, increasing the physical activity. Balloon pulmonary angioplasty is a new highly effective, safe method for treating patients with inoperable CTEPH.

Aim: to assess the state of vascular bed, parenchyma, and perfusion of lungs in patients with chronic thromboembolic pulmonary hypertension (CTEPH) using the method of subtraction computed tomography (CT)., Methods: CT pulmonary angiography (CTPA) was performed in 45 patients with verified CTEPH (18 men, 27 women, age 26-79 years) by CT scanner using the "Lung subtraction" standard protocol. Parameters analyzed were characteristics of the state of main pulmonary artery (MPA) and the right ventricle (RV), and calculated CT angiographic (CTA) obstruction and perfusion defect scores., Results: Significant correlation was found between CTA obstruction score and perfusion defect score (r=0.34, p=0.02). Mean pulmonary arterial pressure (mPAP) correlated with MPA diameter (r=0.4, p=0.02), RV wall thickness (r=0.6, p=0.0003) and the ratio of MPA diameter to ascending aortic diameter (r=0.5, p=0.002). Significant correlation was also found between RV wall thickness and pulmonary vascular resistance (PVR) (r=0.4, p=0.04). Neither CTA obstruction score nor perfusion defect score correlated with PVR and mPAP. The data of CT did not correlate with results of 6-minute walk test., Conclusion: In patients with CTEPH subtraction CTPA allows carrying out complex diagnostics of the state of vascular bed, parenchyma and perfusion of the lungs.

Aim: To evaluate the vascular bed and lung perfusion in patients with chronic thromboembolic pulmonary hypertension (CTEPH) by computed tomography (CT) and to compare the severity of pulmonary arterial (PA) thrombotic lesions concurrent with parenchymal perfusion disorders with angiopulmonographic findings and clinical and hemodynamic characteristics., Subjects and Methods: In the period from November 2015 to May 2016, 22 patients (7 men, 15 women) aged 27 to 67 years with a verified diagnosis of CTEPH were examined using an Aquilion ONE VISION Edition 640 (Toshiba Medical Systems, Japan) CT scanner with a 320-row detector. Perfusion defect in this study was evaluated with the new software allowing one to combine contrast and contrast-free images by a subtraction method. CT data analysis included visual assessment of the vascular bed and lung parenchyma and quantitative assessment with perfusion map construction and semi-automatic determination of the obstruction index (OI) and perfusion defect index (PDI). OI was compared with PDI and mean LA pressure according to the data of right heart catheterization and 6-minute walk test., Results: A statistically significant correlation was found between OI and PDI in patients with CTEPH (Pearson r=0.56; p=0.0065). There were no relationships between mean LA pressure and vascular perfusion indices (OI and PDI) or between vascular perfusion parameters and 6-minute walk test results., Conclusion: CT angiopulmonography in conjunction with pulmonary perfusion assessment (within one study) allows evaluation of the severity of vascular lesions and perfusion disorders and determination of the efficiency of treatment in patients with CTEPH.

The paper gives current approaches to treating chronic thromboembolic pulmonary hypertension (CTEPH) from the document «Federal Guidelines for the Diagnosis and Treatment of CTEPH» approved at the Third Russian Congress on Pulmonary Hypertension on December 11, 2015. The guidelines had been elaborated to optimize the treatment of patients with CTEPH on the basis of an analysis of the data of the present-day registries and multicenter randomized clinical trials, national and international guidelines and consensus documents, and documents published in recent years. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The paper presents indications for and contraindications to pulmonary thromboendartectomy; preparation for surgery; operating room facilities; the specific features of postoperative management and possible complications; and long-term RESULTS: In terms of therapy, in addition to non-pharmacological measures, the authors discuss maintenance and specific treatment options for CTEPH, balloon pulmonary angioplasty, and lung/heart-lung transplantation. In conclusion they propose a management algorithm in patients with CTEPH and requirements for its problem to the center of experts.

Chronic thromboembolic pulmonary hypertension (CTEPH) is precapillary pulmonary hypertension, in which chronic obstruction of large and middle branches of pulmonary arteries (PAs) and secondary changes in the lung microcirculatory bed result in a progressive increase in pulmonary vascular resistance and PA pressure with the development of severe right cardiac dysfunction and heart failure. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The diagnostic criteria for CTEPH are a mean PA pressure of ≥25 mm Hg, as evidenced by right heart catheterization; a PA wedge pressure of ≤15 mm Hg; a pulmonary vascular resistance of >2 Wood units; the presence of chronic/organized thrombi/emboli in the elastic PAs (pulmonary trunk, lobular, segmental, subsegmental PAs); effective anticoagulant therapy at therapeutic dosages over at least 3 months. Up to now, our country has had no guidelines for the diagnosis and treatment of this rare severe disease that, when appropriately untreated, has an extremely poor prognosis. The main task in the preparation of this document was to generalize and analyze the data of current registries, multicenter randomized clinical trials, national and international guidelines, and consensus documents recently published on this problem in order to optimize a diagnostic process and treatment in this category of patients. Part 1 gives a definition of CTEPH, its place in the clinical classification, epidemiology and prognosis, risk factors, pathogenesis and morphology, diagnostic approaches and determination of operability in patients, and specific features of differential diagnosis.