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It is relevant to study of angiogenesis mediators and the mobilization of early endothelial progenitor cells (EPС) from bone marrow into the blood in patients with coronary heart disease (CHD), suffering and not suffering from ischemic cardiomyopathy (ICMP).СHD patients: 30 people with ICMP and 22 people without ICMP, 15 healthy donors. The content of early EPС (VEGFR2+CD34+CD14+) was determined in the blood and bone marrow by flow cytofluorometry, the concentration of MSP-1, SDF-1, VEGF-A – by multiplex analysis, of HIF-1α – by ELISA.Тhe content of SDF-1 and HIF-1α in peripheral blood in patients with CHD without cardiomyopathy was higher than in healthy individuals (respectively 60.00 (50.00-80.00) pg/mL and 6.00 (5.00-6.20) ng/mL versus 30.00 (5.00-45.00) pg/mL, p = 0.049 and 4.60 (3.28-5.11) ng/mL, p = 0.049), with ICMP corresponded to the norm. Тhe concentration of SDF-1 in the bone marrow was higher, and the level of HIF-1α was less than their content in the bloodstream, regardless of the presence of ICMP (respectively 130.0 (90.0-170.0) pg/mL, p = 0.005 and 0.97 (0.80-1.11) ng/mL, p < 0.001). The level of MCP-1 in the blood varied within the normal range in patients with CHD of both study groups (190.0 (168.0-215.0) pg/mL), and in the bone marrow was higher only in patients with ICMP (406.5 (265.0-583.0) pg/mL, p = 0.028). Regardless of ICMP presence, the content of VEGF-A in the blood of patients with CHD corresponded to the norm (3.80 (1.00-6.50) pg/mL) and in myeloid tissue. The number of EPC was increased in the blood of patients with CHD without cardiomyopathy (0.70 (0.46-1.23) and 0.19 (0.13-0.32) %, p < 0.001) and corresponded to their number in the bone marrow. And in patients with ICMP, normal values of the indicator were recorded in the blood with the accumulation of EPC in myeloid tissue (0.57 (0.45-0.98) %, p = 0.019).The development of ICMP is associated with the accumulation of early EPC in myeloid tissue due to their increased retention by an excess of MCP-1 in the bone marrow with weak involvement in the bloodstream due to the lack of a surplus of SDF-1 and HIF-1a in the blood.

Aim. To discuss the functional properties of melatonin which plays a key role in the expression of psychopathological disorders following traumatic brain injury (TBI). The influence of melatonin on neurochemical changes, which may lead to various mental disorders, has been analyzed. Specifically, its impact on asthenia, cognitive impairments, emotional instability, and insomnia, which are among the main symptoms accompanying TBI in the chronic phase, has been examined. The importance of studies on melatonin as a neurotransmitter and its role in the circadian rhythm regulation, which is often impaired as a consequence of TBI, has been emphasized. The mechanisms underlying these disorders may be related to dysfunction of the hypothalamus and interhemispheric interactions. Melatonin may influence the recovery processes after TBI, reducing inflammation and providing optimal conditions for tissue regeneration. It opens up new possibilities for the development of therapy aimed at reducing the impact of trauma consequences on patients. Earlier studies have also indicated the option of using melatonin as an antioxidant, which can protect brain tissue from further damage caused by recovery processes after injury. Studies on pharmacological modulation of the melatonin system could be useful for developing new methods for treatment and prevention of mental disorders associated with TBI. Examining the action of melatonin receptor antagonists and agonists can help to choose optimal treatment strategies aimed at correcting disorders arising as a result of trauma. It holds promise for faster and more complete recovery of mental health in TBI patients. Conclusions. Considering the significance of melatonin in regulation of physiological processes, further studies may enrich understanding of its role, ensuring the development of more effective treatment methods. Given the safe profile of melatonin and its availability as a supplement, supportive studies in this direction have the potential to positively impact clinical practice.

Background. In the pathogenesis of ischemic cardiomyopathy (ICMP), angiopoiesis remains unexplored.The aim. To describe the vasculature of the heart and the imbalance of angiogenesis mediators in the coronary circulation in association with the number of endothelial progenitor cells (EPC) and desquamated endothelial cells (DEC) in the blood of patients with coronary heart disease (CHD), suffering and not suffering from ICMP.Methods. Fifty-two patients with CHD (30 patients with ICMP, 22 patients without ICMP), 15 healthy donors were examined. The content of EPC (CD14+CD34+VEGFR2+) in the blood from the cubital vein and DEC (CD45–CD146+) in the blood from the coronary sinus and the cubital vein was determined by flow cytometry. The concentrations of VEGF-A (vascular endothelial growth factor A), PDGF (platelet-derived growth factor), and SDF-1 (stromal cell-derived factor 1) in blood plasma were recorded using immunofluorescence assay; the angiopoietin-2, MMP-9 (matrix metallopeptidase 9) were recorded using enzyme immunoassay. In myocardial biopsies the specific area of vessels and the expression of αSMA (smooth muscle alpha-actin) were determined by morphometric and immunohistochemical methods.Results. In the peripheral blood of patients with CHD, regardless of the presence of ICMP, the DEC content exceeded the physiological level, and the VEGF-A, PDGF, angiopoietin-2, and MMP-9 corresponded to the norm. In CHD patients without cardiomyopathy, there was an excess of SDF-1 and EPC in the blood from the cubital vein, and in ICMP, their physiological significance was noted. In the coronary blood flow in patients with CHD without cardiomyopathy, an increase in the concentration of PDGF was found, which was not determined in patients with ICMP, who had an increased content of DEC, angiopoietin-2 and MMP-9. The specific area of the vessels in the patients of the two groups was comparable; the expression of αSMA in ICMP was 6.2 times lower than in patients with CHD without cardiomyopathy.Conclusion. The development of ICMP is accompanied by impaired maturation of vessels in the myocardium, associated with the absence of a compensatory reaction of activation of cellular and humoral factors of angiogenesis.

Relevance. Intrapersonal emotional competence - the ability to recognize, understand and regulate one's own emotional states - is traditionally considered as a factor in both the ontogenetic development of empathy and its situational manifestation. However, there is a question of different levels and nature of the sources of emotional competence and empathy deficits and, accordingly, different ways to compensate for them, which is of particular importance for the practice of developing empathy, including the professional context of psychological counseling and psychotherapy. Goal. The purpose of this study was to analyze the diversity of connections between empathy, alexithymia as an indicator of the deficit of emotional competence in relation to one's own feelings and psychological mindedness as an indicator of intentionality in relation to feelings and experiences. Materials and methods. On a combined sample of 1,123 people, including representatives of three cultures - Russia, Belarus, Armenia - data from the IRI questionnaire (empathy), TAS–20 (alexithymia) and «The psychological mindedness scale» were collected and analyzed. Results. The absence of significant negative connections of empathy with difficulties of awareness and identification of feelings, a stable significant positive connection with them of empathic personal distress, as well as externally oriented thinking with low empathy were found. 5 clusters have been identified, reflecting different variants of the connection between emotional competence and empathy. Conclusions. The conclusion is made about the relative independence of the influence of intentionality and competence/lack of emotional competence on empathy, as well as the need for further analysis of intentionality in the field of experiences and emotions.

Introduction/aimsSevere acute respiratory syndrome coronavirus-2 2019 (SARS-CoV2/COVID-19) is frequently more severe in individuals with pre-existing respiratory and cardiovascular conditions. The impact on patients with neuromuscular disorders is of concern, but remains largely unknown. Late-onset Pompe disease (LOPD) is a lysosomal-storage disorder characterized by progressive skeletal and respiratory muscle degeneration. Mortality is typically caused by respiratory failure. We examined the impact of COVID-19 on these patients.MethodsThis is a case series of four patients with LOPD who contracted COVID-19.ResultsAll patients had a mild/moderate illness from COVID-19 and did not require hospitalization. Neurological worsening occurred in one, with no change in physical ability in the other three, and respiratory symptoms remained stable in all four.DiscussionCOVID-19 infection can result in a benign course in some patients with LOPD. However, individuals with LOPD remain at high risk and should receive COVID-19 vaccinations and exercise precautions to avoid exposure to COVID-19 infection.

Background. Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease; it is characterized by left ventricular (LV) hypertrophy that cannot be explained by hemodynamic causes. It is believed that sarcomere dysfunction underlies the pathogenesis of this disease, however, only half of patients with the HCM phenotype have mutations in sarcomere-encoding genes. HCM is distinguished by both high genetic and clinical heterogeneity and therefore more studies are seeking to investigate a regulation of gene expression in HCM and how the abnormalities in this process can affect disease phenotype. One of the levels of regulation of gene expression a post-transcriptional level is mediated by short non-coding microRNAs that inhibit protein synthesis. Aim. To identify the correlations between levels of circulating microRNAs, previously shown to be associated with HCM, and clinical parameters of HCM patients. Materials and methods. Correlation analysis of miR-499a-5p, miR-454 and miR-339-5p plasma levels and clinical parameters of 33 HCM patients, examined from 2019 to 2021, has been performed. Results. Variants in HCM-associated genes were found in 49% of patients. There were no clinical differences between genotype-positive and genotype-negative patients. MiR-499a-5p level correlated with LV ejection fraction, miR-454 level with LV diastolic function parameters and miR-339-5p level with left atrium dimension. Conclusion. Levels of certain circulating microRNAs correlate with echocardiographic parameters in HCM patients.

Transthyretin amyloidosis (ATTR-amyloidosis) is a systemic disorder associated with extracellular deposition in the tissues and organs of amyloid fibrils, transthyretin-containing insoluble protein-polysaccharide complexes. The change in transthyretin conformation, leading to its destabilization and amyloidogenicity, can be acquired (wild type, ATTRwt) and hereditary due to mutations in the TTR gene (variant, ATTRv) [1, 2]. Hereditary ATTR-amyloidosis has an earlier onset and greater phenotypic diversity. The age of the manifestation, the predominant phenotype, and the prognosis are often determined by the genetic variant. To date, more than 140 variants in the TTR gene have been identified; however, most of them are described in single patients and do not have clear evidence of pathogenicity. The prospects of a new pathogenetic treatment of ATTR-amyloidosis [3], especially effective in the early stages of the disease, increases the relevance of timely diagnosis, which is challenging due to physicians' lack of awareness. This article presents a clinical case of ATTRv-amyloidosis associated with a rare pathogenic variant in the TTR gene and a newly described skin symptom. This article is a literature review.

The research is devoted to the theoretical and applied organizational bases to held of the comparative analysis of the economic development of the regions of the Black Sea region. The main purpose of the article is the process of comparative analysis of economic development of the Black Sea region. The article tests the authors' hypothesis about the adequacy of the indicators defined for evaluation through the proposed number of relative indicators, which in the complex will characterize the achievements of the region in ensuring the economic stability of the regional system, quality of transformation processes and indirectly the conditions created by public authorities for economic development. There is confirmed the dependence of the use of the proposed methodological approaches and the constructed comparative profile of the regions of the region, which can be useful for identifying the strengths and weaknesses of the region, outlining key issues and developing regional development plans and programs. It is determined that the largest vector length in the Mykolaivska region, which indicates that in the region on a number of economic indicators achieved higher results than in other regions of the Black Sea region and on average in other regions of Ukraine during the study period.

The aim of the study was to evaluate the expression of scavenger receptors (CD163, CD204, CD206) on macrophages in patients with pulmonary tuberculosis, depending on the clinical form of the disease and sensitivity of the pathogen to anti-tuberculosis drugs.Materials and methods. 64 patients with pulmonary tuberculosis (TB) were examined: 26 patients with disseminated pulmonary tuberculosis (DTB) and 38 patients with infiltrative pulmonary tuberculosis (ITB). Of these, 42 patients secreted Mycobacterium tuberculosis (MBT) sensitive to basic antituberculosis drugs (ATBD), and 22 patients secreted MBT resistant to first-line anti-TB drugs. Material for the study was venous blood. To isolate monocytes from the whole blood in order to transform them into macrophages, Ficoll density gradient centrifugation with a density of 1.077 g / cm3 was used followed by immunomagnetic separation of CD14+ cells. Monocytes were cultured in the X-VIVO 10 medium with gentamicin and phenol red with the addition of macrophage colony-stimulating factor (M-CSF) (5 ng / ml) at a concentration of 1×106 cells / ml with stimulators: interleukin (IL)-4 (10 ng / ml) and interferon (IFN) γ (100 ng / ml). Immunophenotyping of macrophages was performed using monoclonal antibodies to CD163, CD204, and CD206 on the Beckman Coulter CytoFLEX LX Flow Cytometer. The analysis of the obtained data was carried out using the CytExpert 2.0 software. The results were analyzed using statistical methods.Results. Switching the phenotype of macrophages from the M1-like proinflammatory phenotype to M2-like antiinflammatory one contributes to the chronic course of pulmonary TB, dissemination, and persistence of infection. In the present study, we analyzed the features of the expression of CD163, CD204, and CD206 scavenger receptors on macrophages in patients with pulmonary TB. An increase in the number of macrophages carrying markers of the M2 subpopulation (CD163, CD204, and CD206) on their surface was noted, regardless of the clinical form of pulmonary TB and drug resistance of M. tuberculosis.Conclusion. Studying the mechanisms underlying M1 or M2 activation of macrophages is necessary for a deeper understanding of the immunopathogenesis of TB and the role of innate immunity cells in protecting the body from mycobacteria. The analysis of the expression of scavenger receptors CD163, CD204, and CD206 on macrophages allowed to conclude that, in pulmonary TB, especially in patients with drug resistant M. tuberculosis and infiltrative TB, regulatory mechanisms that suppress the activation of innate immunity are implemented together with polarization of macrophage differentiation towards the M2 phenotype. It may be the cause of immune deficiency induced by the pathogen.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease associated with morbidity and mortality at any age. As studies in recent decades have shown, the genetic architecture of HCM is quite complex both in the entire population and in each patient. In the rapidly advancing era of gene therapy, we have to provide a detailed molecular diagnosis to our patients to give them the chance for better and more personalized treatment. In addition to emphasizing the importance of genetic testing in routine practice, this review aims to discuss the possibility to go a step further and create an expanded genetic panel that contains not only variants in core genes but also new candidate genes, including those located in deep intron regions, as well as structural variations. It also highlights the benefits of calculating polygenic risk scores based on a combination of rare and common genetic variants for each patient and of using non-genetic HCM markers, such as microRNAs that can enhance stratification of risk for HCM in unselected populations alongside rare genetic variants and clinical factors. While this review is focusing on HCM, the discussed issues are relevant to other cardiomyopathies.

Background. Angiogenic endothelial dysfunction and progenitor endothelial cells (EPCs) in ischemic cardiomyopathy (ICMP) have not been studied enough.The aim. To establish the nature of changes in the cytokine profile and HIF-1α in blood and bone marrow associated with impaired differentiation of monocytic progenitor cells of endotheliocytes (CD14+VEGFR2+) in the bone marrow and their migration into the blood in patients with coronary heart disease (CHD), suffering and not suffering from ICMP.Materials and methods. A single-stage, single-centre, observational case-control study was conducted involving 74 patients with CHD, suffering and not suffering from ICMP (30 and 44 people, respectively), and 25 healthy donors. In patients with CHD, bone marrow was obtained during coronary bypass surgery, peripheral blood – before surgery. Healthy donors were taken peripheral blood. The number of CD14+VEGFR2+ in bone marrow and blood was determined by flow cytometry; the concentration of IL-6, TNF-α, M-CSF, GM-CSF, MCP-1 and HIF-1α – by the method of enzyme immunoassay.Results. A high content of CD14+VEGFR2+ cells in the blood of patients with CHD without cardiomyopathy was established relative to patients with ICMP against the background of a comparable number of these cells in myeloid tissue. Regardless of the presence of ICMP in the blood, patients with CHD showed an excess of TNF-α, a normal concentration of IL-6, GM-CSF, HIF-1α and a deficiency of M-CSF, and in the bone marrow supernatant, the concentration of IL-6 and TNF-α exceeded that in the blood plasma (the level of GM-CSF – only in patients without cardiomyopathy). With ICMP, the normal concentration of MCP-1 was determined in the blood plasma, and with CHD without cardiomyopathy, its elevated content was determined.Conclusion. The formation of ICMP is accompanied by insufficient activation of EPCs migration with the CD14+VEGFR2+ phenotype in blood without disruption of their differentiation in the bone marrow, which associated with the absence of an increase in the concentration of MCP-1 in blood plasma and not associated with the plasma content of M-CSF, GM-CSF, HIF-1α, IL-6 and TNF-α.

Modern Agricultural production is characterized by an increased need for specialized, highly productive, complementary varieties of feed crops adapted to specific natural and climatic conditions, grown using various technologies, for certain groups of animals. Among the perennial legumes in the south of Russia, sainfoin is in special demand. The purpose of the current study was to estimate the variety samples of various types of sainfoin and a new variety of Transcaucasian sainfoin ‘Kravtsov’ according to the main economically valuable traits and properties when used for feed and seeds. The study was carried out in the zone of unstable moisture in the Stavropol Territory. The soil of the experimental field was represented by low-humus micellar-carbonate medium loamy chernozem, with 3.2 % humus, 12 mg/kg phosphorus and 260 mg/kg potassium in the arable layer. The prevailing weather conditions during the research period made it possible to give a comprehensive estimation of the studied material. According to the results of Competitive Variety Testing, a variety sample of Transcaucasian sainfoin was selected from the studied three types of the crop, which, due to the results of the State Variety Testing in 2021, was included in the State List of Breeding Achievements of the Russian Federation with permission to use in all regions of the country. A new variety of Transcaucasian sainfoin ‘Kravtsov’ has a complex of such economically valuable traits as intensive spring and post-harvest regrowth, high foliage (42–45 % in the first cut, 52–57 % in the second cut), and resistance to unfavorable environmental factors. The plant height in the budding phase was 110–115 cm, which exceeded standard plants’ height by 10–15 %. The variety is highly resistant to drought and lodging, tolerant to diseases and pests. The variety is highly productive with 33.8 t/ha of green mass, 9.7 t/ha of hay and 1.1 t/ha of seeds. There is 17% crude protein in dry matter. 1000-seed weight is 23–25 g.

Aim. To identify disturbances of differentiation and subpopulation composition of VEGFR2+ cells in the blood and bone marrow associated with the features of the cytokine profile in the blood and bone marrow in patients with coronary artery disease (CAD) with and without ischemic cardiomyopathy (ICM).Materials and methods. The study included 74 patients with СAD with and without ICM (30 and 44 people, respectively) and 18 healthy donors. In all patients with СAD, peripheral blood sampling was performed immediately before coronary artery bypass grafting, and bone marrow samples were taken during the surgery via a sternal incision. In the healthy donors, only peripheral blood sampling was performed. In the bone marrow and blood samples, the number of VEGFR2+ cells (CD14+VEGFR2+ cells) and their immunophenotypes CD14++CD16-VEGFR2+, CD14++CD16+VEGFR2+, CD14+CD16++VEGFR2+, and CD14+CD16-VEGFR2+ was determined by flow cytometry. Using enzyme-linked immunosorbent assay, the levels of VЕGF-А, TNFα, M-CSF, and IL-13, as well as the content of MCP-1 (only in the blood) and the M-CSF / IL-13 ratio (only in the bone marrow) were determined.Results. The content of CD14+VEGFR2+ cells in the blood of CAD patients with and without ICM was higher than normal values due to the greater number of CD14++CD16-VEGFR2+, CD14++CD16+VEGFR2+, and CD14+CD16++VEGFR2+. In the bone marrow of the patients with ICM, the content of CD14++CD16-VEGFR2+, CD14+CD16++VEGFR2+, and CD14+CD16-VEGFR2+ was lower than in patients with CAD without ICM, and the number of CD14++CD16+VEGFR2+ cells corresponded to that in the controls. Regardless of the presence of ICM in CAD, a high concentration of TNFα and normal levels of VEGF-A and IL-13 were observed in the blood. In CAD without ICM, an excess of MCP-1 and deficiency of M-CSF were revealed in the blood. In the bone marrow, the levels of VEGF-A, TNFα, M-CSF, and IL-13 were comparable between the groups of patients against the background of a decrease in the M-CSF / IL-13 ratio in the patients with ICM.Conclusion. Unlike CAD without cardiomyopathy, in ICM, no excess of VEGFR2+ cells and MCP-1 in the blood is observed, which hinders active migration of CD14+CD16++VEGFR2+ cells from the myeloid tissue, and a decrease in the M-CSF / IL-13 ratio in the bone marrow disrupts differentiation of other forms of VEGFR2+ cells, preventing vascular repair.

Abstract Aims To describe the natural history of SARS‐CoV‐2 infection in patients with hypertrophic cardiomyopathy (HCM) compared with a control group and to identify predictors of adverse events. Methods and results Three hundred and five patients [age 56.6 ± 16.9 years old, 191 (62.6%) male patients] with HCM and SARS‐Cov‐2 infection were enrolled. The control group consisted of 91 131 infected individuals. Endpoints were (i) SARS‐CoV‐2 related mortality and (ii) severe clinical course [death or intensive care unit (ICU) admission]. New onset of atrial fibrillation, ventricular arrhythmias, shock, stroke, and cardiac arrest were also recorded. Sixty‐nine (22.9%) HCM patients were hospitalized for non‐ICU level care, and 21 (7.0%) required ICU care. Seventeen (5.6%) died: eight (2.6%) of respiratory failure, four (1.3%) of heart failure, two (0.7%) suddenly, and three (1.0%) due to other SARS‐CoV‐2‐related complications. Covariates associated with mortality in the multivariable were age {odds ratio (OR) per 10 year increase 2.25 [95% confidence interval (CI): 1.12–4.51], P = 0.0229}, baseline New York Heart Association class [OR per one‐unit increase 4.01 (95%CI: 1.75–9.20), P = 0.0011], presence of left ventricular outflow tract obstruction [OR 5.59 (95%CI: 1.16–26.92), P = 0.0317], and left ventricular systolic impairment [OR 7.72 (95%CI: 1.20–49.79), P = 0.0316]. Controlling for age and sex and comparing HCM patients with a community‐based SARS‐CoV‐2 cohort, the presence of HCM was associated with a borderline significant increased risk of mortality OR 1.70 (95%CI: 0.98–2.91, P = 0.0600). Conclusions Over one‐fourth of HCM patients infected with SARS‐Cov‐2 required hospitalization, including 6% in an ICU setting. Age and cardiac features related to HCM, including baseline functional class, left ventricular outflow tract obstruction, and systolic impairment, conveyed increased risk of mortality.

A.S. Panchenko, E.V. Bem, G.N. Chumakova, I.V. Myznikova St. Petersburg State Pediatric Medical University, St. Petersburg, Russian Federation Cytomegalovirus infection (CMVI) is the most common viral infection in childhood. Congenital CMVI is quite well studied, always causes alertness of neonatologists, and has a diverse clinical picture after the birth of a child. Certain difficulties in diagnosis are noted in children who have postnatal CMVI. As a rule, the infection is asymptomatic or slightly symptomatic and has no special serious clinical consequences, unlike congenital CMVI, which causes hearing loss and central nervous system damage. It is known that postnatal infection is dangerous for children with impaired immune system. This risk group consists of preterm infants, mainly with very low and extremely low body weight, and full-term infants with severe concomitant congenital and/or acquired pathology. The question of infecting ways for children with postnatal CMVI remains debatable. The article also discusses the various clinical manifestations of postnatal CMVI, which can occur according to the type of CMV-related sepsis-like syndrome, hepatitis, diarrhea, neutropenia. The article highlights the importance of clear laboratory diagnostic criteria during establishing the diagnosis. There are always a lot of questions from the doctor regarding the prescription of specific treatment in CMVI and prevention methods of postnatal CMVI in full-term infants. This article also presents clinical case of postnatal CMVI in full-term infants with combined pathology receiving treatment in the conditions of the Perinatal center of St. Petersburg State Pediatric Medical University. Keywords: postnatal cytomegalovirus infection, cytomegalovirus hepatitis, hearing loss, diagnosis, treatment, breast milk, neonates. For citation: Panchenko A.S., Bem E.V., Chumakova G.N., Myznikova I.V. Сourse patterns of postnatal cytomegalovirus infection in full-term infants. Cases in clinical practice. Russian Journal of Woman and Child Health. 2023;6(2):206–214 (in Russ.). DOI: 10.32364/2618-8430-2023-6-2-206-214.

Hypertrophic cardiomyopathy (HCM) is a common inherited cardiac disorder characterized by marked clinical and genetic heterogeneity. Ethnic groups underrepresented in studies may have distinctive characteristics. We sought to evaluate the clinical and genetic landscape of Russian HCM patients. A total of 193 patients (52% male; 95% Eastern Slavic origin; median age 56 years) were clinically evaluated, including genetic testing, and prospectively followed to document outcomes. As a result, 48% had obstructive HCM, 25% had HCM in family, 21% were asymptomatic, and 68% had comorbidities. During 2.8 years of follow-up, the all-cause mortality rate was 2.86%/year. A total of 5.7% received an implantable cardioverter-defibrillator (ICD), and 21% had septal reduction therapy. A sequencing analysis of 176 probands identified 64 causative variants in 66 patients (38%); recurrent variants were MYBPC3 p.Q1233* (8), MYBPC3 p.R346H (2), MYH7 p.A729P (2), TPM1 p.Q210R (3), and FLNC p.H1834Y (2); 10 were multiple variant carriers (5.7%); 5 had non-sarcomeric HCM, ALPK3, TRIM63, and FLNC. Thin filament variant carriers had a worse prognosis for heart failure (HR = 7.9, p = 0.007). In conclusion, in the Russian HCM population, the low use of ICD and relatively high mortality should be noted by clinicians; some distinct recurrent variants are suspected to have a founder effect; and family studies on some rare variants enriched worldwide knowledge in HCM.