Eduard G Malev

Federal Almazov Medical Research Centre · Connective tissue disorders

The aim of the study is to determine the impact of the underlying etiology (Barlow's disease or fibroelastic deficiency) on left ventricular function in patients with degenerative mitral valve disease and severe mitral regurgitation. We studied 233 patients (mean age: 53.8 ± 12.9) undergoing surgery for severe mitral regurgitation due to degenerati...

Background: In some inherited connective tissue diseases with involvement of the cardiovascular system, for example, Marfan syndrome, early impairment of left ventricular function, which have been described as Marfan-related cardiomyopathy has been reported. Our aim was to evaluate the left ventricular function in young adults with mitral valve pr...

Objective: To evaluate left ventricular function in young adults with mitral valve prolapse (MVP) without significant mitral regurgitation using two-dimensional strain imaging. Methods and results: A total of 58 asymptomatic young subjects (mean [± SD] age 19.7±1.6 years; 72% male) with MVP were compared with 60 sex- and age-matched healthy subj...

Aim To evaluate the effect of mitral valve (MV) repair and replacement on the incidence of ventricular arrhythmias (VA) and to identify risk factors for the persistence of VA in patients with MV prolapse and severe mitral regurgitation (MR) during a mid-term follow-up. Material and methods A single-site observational, prospective study successively...

Valvular heart disease remains one of the causes of cardiovascular morbidity and mortality worldwide. Aortic stenosis is the most common valvular pathology requiring cardiac surgery. For elderly and senile patients with high risks of volumetric cardiac surgery, a new type of biological prosthesis, a transcatheter implantable aortic valve, has becom...

The speckle tracking strain is becoming a frequently used marker of subclinical left ventricular systolic dysfunction. Despite the wide range of data concerning left ventricular strain variability in the general population and its changes in various pathologic conditions, the information about the impact of medical therapy on left ventricle strain...

Background: Different imaging techniques, such as echocardiography (ECHO) and CT, allow to assess aortic stenosis (AS) severity and could be used to study its progression. But only PET/CT open opportunities to assess activity of valvular inflammation and calcification in vivo . The aim of this study was to assess prognostic value of valvular inflam...

The review article presents modern concepts of the mitral valve prolapse. The issues of pathogenesis, diagnostic criteria of primary and probable prolapse are reflected, the issues of epidemiology are considered in detail. The problems associated with significant overdiagnosis of this condition in our country (up to 36.8%) are noted, which is due t...

Growing evidence supports role transforming growth factor-β (TGF-β) as a mediator of the myxomatous changes in the mitral valve prolapse. Recently, angiotensin II receptor blockers (ARB) emerged as a potentially effective inhibitor of TGF-β signaling. Treatment of valvular interstitial cells (VICs) with ARB resulted in effective inhibition of TGF-β...

Introduction Transforming growth factor-β1 (TGF-β1) is a crucial regulatory cytokine that contributes to the development of the mitral valve and regulates extracellular matrix protein synthesis in syndromic and non-syndromic mitral valve prolapse (MVP). Our aim was to evaluate the effect of elevated circulating TGF-β level on the progression of the...

Valvular heart disease is widespread among general population, mainly in older age groups, who often require noncardiac surgery. As the population ages, the significance of this problem will only increase. The basic principles of perioperative managing such patients by a multidisciplinary team, necessarily including a cardiologist, are systematized...

Aim . To compare markers of high cardiovascular risk and stress echocardiography results depending on the type of blood pressure (BP) response to exercise in patients without obstructive coronary artery disease. Material and methods . Our single-center cross-sectional study included 96 patients without hemodynamically significant coronary artery st...

Aim To determine diagnostic capabilities of the expanded protocol for stress echocardiography (stress-EchoCG) with comprehensive evaluation of clinical and echocardiographic indexes in differential diagnosis of dyspnea. Material and methods This study included 243 patients (123 women and 120 men) who were referred to outpatient stress-EchoCG during...

Funding Acknowledgements Type of funding sources: None. Background. The positive effect of blood pressure (BP) lowering medications on global longitudinal strain (GLS) and left ventricle (LV) dyssynchrony even in patients with narrow QRS complexes at rest has been established at rest. The dynamic changes of GLS and dyssynchrony markers on exertion...

Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Grant of Russian Foundation for Basic Research Introduction Long existing pressure overload results in left ventricular (LV) hypertrophy in severe aortic stenosis (AS). Post-TAVI left ventricular remodeling after relief of the high-pressure overload could lead to...

Funding Acknowledgements Type of funding sources: None. Introduction Improvement in malignant ventricular arrhythmias (VA) has been reported after mitral valve surgery in some mitral valve prolapse patients (MVP) with severe degenerative mitral regurgitation. Mitral annular disjunction, posterior systolic curling, and mitral annular abnormal contr...

Funding Acknowledgements Type of funding sources: None. Background Hypertensive response to exercise (HRE) has negative prognostic value but its impact on the left ventricle (LV) contractility and on stress echocardiography (SE) results remains controversial. The global longitudinal strain (GLS) and LV dyssynchrony changes in response to afterload...

Background Left ventricular (LV) remodeling could be found in a range of connective tissue disorders with involvement of transforming growth factor (TGF)-β pathway. Only rare data are available on it in patients with mitral valve prolapse (MVP). The aim of the study is to assess mechanisms of myocardial remodeling and diastolic dysfunction in asymp...

Background. Hypertensive response of blood pressure (BP) to exercise has substantial prognostic impact. Hypertensive response to exercise is shown to be a predictor for development of hypertension and stroke. The data concerning hypertensive response to exercise influence on stress echocardiography results are controversial. Objective. The aim of t...

Introduction. Small heart anomalies (SHA) are the morphological basis for functional changes in cardiac activity and can exacerbate the course of organic heart lesions. The most studied SHA include false chords of the left ventricle (FCLV) and mitral valve prolapse. Prevalence, association with external signs of dysembryogenesis, as well as the pre...

Assessment of systolic and diastolic ventricular function is one of the main goals of echocardiography. Nevertheless, some patients require data beyond standard echocardiographic protocol for making more precise clinical decision and prognosis determination. The spectrum of novel parameters, including myocardial strain and strain rate, provides mor...

For many hereditary connective tissue disorders (HCTD), especially Marfan syndrome, remodeling of the heart and main vessels is described, which is manifested by a decrease in the systolic function of the left ventricle and expansion of the thoracic aorta. Evaluation of morphometric characteristics of the heart and main vessels in patients with oth...

Purpose Current understanding of the pathogenesis of thoracic aortic aneurysm (TAA) in Marfan syndrome (MS) focuses upon abnormal activity of the transforming growth factor beta (TGF-β) signalling pathway. Circulating TGF-β predicts cardiovascular events in patients with MS and is elevated in the entire spectrum of aortic syndromes. Marfanoid habit...

Introduction There is limited data on the efficacy of surgical repair in reducing ventricular arrhythmia (VA) in mitral valve prolapse (MVP) patients. Improvement in malignant ventricular arrhythmias has been reported only in isolated cases after mitral valve surgery. Our aim was to evaluate the possible effects of mitral valve repair on left ventr...

Aim . To determine the inflammation and calcification activity in aortic stenosis (AS) by assessing the accumulation of ¹⁸ F-FDG and ¹⁸ F-NaF in the aortic valve; to study the relationship of the AS severity, aortic calcification and the accumulation of ¹⁸ F-FDG and ¹⁸ F-NaF. Material and methods . The study included 62 patients with asymptomatic A...

According to contemporary views, hereditary connective tissue disorders divided classified Marfan syndrome, Loeys-Dietz’s, Ehlers-Danlos syndrome, the primary mitral valve prolapse. It is known that the fibrillinopaty, which include the Marfan syndrome and Loeys-Dietz’s is characterized by activation of TGF-β signaling pathway. With high le vels of...

Marfan syndrome is a hereditary, autosomal disease with a relatively high prevalence in the general population. Numerous manifestations of Marfan syndrome are expressed in a wide range of disorders involving cardiovascular, ocular and musculoskeletal systems. The article describes the principles of management of the syndrome in children and adolesc...

Transforming growth factor-β (TGF-β) is a multifunctional cytokine with fibrogenic properties. Also increased TGF-β signaling contributes to the development of myxomatous changes of the mitral valve (MV). Recent data suggests that TGF-β may play a potentially important role in the heart arrhythmogenesis. Our aim was to evaluate the possible proarrh...

Introduction Calcification is the key pathological process in aortic stenosis (AS) development. Circulating biomarkers and nuclear imaging methods could characterize the valvular calcification activity. Purpose To compare diagnostic values of serum fetiun A level and PET/CT with 18F NaF in assessment of aortic valve calcification. Methods 88 pts...

Background: Ascending aortic (AA) dilatation is common in patients with bicuspid aortic valve (BAV). In BAV replacement, surgery of the AA is indicated in the case if AA diameter exceeds 45 mm. Aortic valve replacement combined with an AA intervention is associated with increased risk of complications. The feasibility of the reduction ascending aor...

Aneurysm of the thoracic aorta of any origin is traditionally considered a pathology for surgical correction. Traditionally the patients are referred for the surgery (prosthetics or endovascular treatment) when thoracic aorta diameter achieves 50–55 mm. However, the management strategy and conservative treatment in case of the smaller aorta dilatio...

Background: In Marfan syndrome early impairment of left ventricular systolic function has been reported. Our aim was to evaluate the left ventricular systolic function in young adults with Marfanoid habitus (MH) (includes arachnodactylia, dolichostenomelia, high palate, deformations of the thorax). Methods: We studied 137 young subjects (mean ag...

The article describes the current algorithms of diagnostics of inherited (dysplasia) of connective tissue (DCD) that are currently in force in the territory of the Russian Federation, compared in terms of identification of morpho-functional characteristics of heart. Describes the shortcomings of these algorithms do not take into account the gender...

To study the possibility of using thrombin generation tests in platelet-rich and platelet-poor plasma for evaluation of dual antiplatelet therapy efficacy in patients with coronary artery disease (CAD), following percutaneous coronary intervention. Venous blood was analyzed from CAD patients aged 53-75 years who had undergone percutaneous coronary...

Official Russian guidelines for inherited connective tissue diseases distinguish a range of dysplastic phenotypes, with the marfanoid habitus being one of them. The prevalence of the marfanoid habitus and morfometrical heart singularities among the young practically healthy persons were not studied before. Materials and methods of the study: 560 pr...

Marfan syndrome is a common genetically determined pathology of connective tissue. It was showed a reduction in systolic and diastolic left ventricular function in patients with Marfan syndrome, as well as the increase in left ventricle size, regardless of previous surgical intervention. Now in literature use the term “cardiomyopathy in Marfan synd...

The article focuses on the review of the main methods of aorta visualization. The methods are described, as the algorithm of investigation method selection, strategy of patient’s management with aorta dilation. Special attention is paid for the patients with genetic disorders that may cause aorta dilation.

Aim. To estimate the prevalence of disordered repolarization and its relation with the character of vegetative dysfunction in younger persons with MP and MVP. Material and methods. Totally 285 persons studied of the young age (mean age 19,4+1,4 y.). Phenotypical, anthropometric and clinical investigation performed, ECG, EchoCG, Holter monitoring (H...

Aim. To evaluate systemic involvement of connective tissue (SICT) in young adults with mitral valve prolapse (MVP) without significant mitral regurgitation (MR). Material and methods. We studied 78 asymptomatic young subjects (mean age 19,7±1,6, 72% male) with MVP in comparison with 80 sex- and age-matched healthy subjects. We performed phenotypic...

Aim. To estimate the prevalence of disordered repolarization and its relation with the character of vegetative dysfunction in younger persons with MP and MVP. Material and methods. Totally 285 persons studied of the young age (mean age 19,4+1,4 y.). Phenotypical, anthropometric and clinical investigation performed, ECG, EchoCG, Holter monitoring (H...

The article focuses on the review of the main methods of aorta visualization. The methods are described, as the algorithm of investigation method selection, strategy of patient’s management with aorta dilation. Special attention is paid for the patients with genetic disorders that may cause aorta dilation.

A 33-year-old woman underwent successful vaginal delivery despite previously unsuspected 8-cm ascending and 6-cm descending aortic aneurysms. These were repaired immediately after delivery.

We thank to Dr Yiğiner and colleagues for the interest in our study regarding the left ventricular function in young adults with mitral valve prolapse without significant mitral regurgitation1. In their commentary were emphasized the importance of the etiological distinction of mitral valve prolapse for medium and long-term prognosis and reconstruc...

Purpose: The inhibition by angiotensin II receptor blockers (ARB) the transforming growth factor-b (TGF-b) induced extracellular matrix production has been shown recently in cultured valvular interstitial cells obtained from patients with mitral valve prolapse (MVP) undergoing mitral valve repair. Our aim was to evaluate the effect of ARB therapy o...

Purpose: Increased transforming growth factor-β (TGF-β) signaling contribute to the development of thoracic aortic aneurism (TAA) in Marfan syndrome, Loeys-Dietz syndrome, and myxomatous changes in mitral valve prolapse (MVP). But there are novel data seem to suggest that loss-of-function mutations in a gene encoding TGF-β2 paradoxically cause a sy...

Purpose: Medial degeneration is a main pathology associated with Thoracic Aortic Aneurysm (TAA). But there are data seem to suggest that TAA in Bicuspid (BAV) and Tricuspid Aortic Valve (TAV) patients arises from different molecular, cellular and genetic mechanisms. The aim of the study was to assess histological findings in thoracic aortic aneurys...

Ghent nosology (2010) propose the score of connective tissue Systemic Involvement (SI). The majority of SI signs are represented by the skeletal features. We suppose, that the high score of SI typical for Marfanoid Habitus (MH) and Mitral Valve Prolapse (MVP) could indicate the damage of the heart extracellular matrix and serve as a cause of the Ca...

Purpose: Ventricular arrhythmias are known to be common in symptomatic patients with Mitral Valve Prolapse (MVP). The aim of our study was to investigate the prevalence, echocardiographic and biochemical predictors, and possible mechanisms of ventricular arrhythmias in young asymptomatic patients with MVP without significant mitral regurgitation. M...

Purpose. Medial degeneration is a main pathology associated with Thoracic Aortic Aneurysm (TAA). But there are data seem to suggest that TAA in Bicuspid (BAV) and Tricuspid Aortic Valve (TAV) patients arises from different molecular, cellular and genetic mechanisms. The aim of the study was to assess histological findings in thoracic aortic aneurys...

The paper reviews general factors underlying the variety of clinical manifestations of inherited connective tissue disorders (ICTD). The authors make an attempt to harmonise the existing ICTD terminology with the standard international classification. It is proposed to use the Russian term “dysplasia” as a synonym of ICTD and to divide all ICTD int...

Purpose: Ventricular arrhythmias are known to be common in symptomatic patients with mitral valve prolapse (MVP). The aim of our study was to investigate the prevalence, echocardiographic and biochemical predictors, and possible mechanisms of ventricular arrhythmias in young asymptomatic patients with MVP without significant mitral regurgitation. M...

Objective: Transforming growth factor-β (TGF-β) is a multifunctional cytokine with fibrogenic and hemodynamic properties. It has been demonstrated that endorgan damage in arterial hypertension and blood pressure (BP) itself has been related to increased circulating levels of TGF-β. Also increased TGF-β signaling contribute to the development of myx...

Objective: Inflammation via activation of the cardiac angiotensin II system would play a role in the aggravation of left ventricular (LV) remodeling and dysfunction in hypertensive patients. Also inflammation may contribute to the pathogenesis of mitral valve prolapse (MVP). We investigated impact of interleukins (IL) and chemokines on LV remodelin...

Aim. To assess the severity and potential mechanisms of left ventricular (LV) myocardial remodelling and diastolic dysfunction in symptom-free young patients with mitral valve prolapse (MVP), but no arterial hypertension or significant mitral regurgitation. Material and methods. The study included 78 patients with MVP (mean age 19,7±1,6 years; 72%...

Aim. To assess autonomous regulation status in young patients with mitral valve prolapse (MVP), Marfanoid habitus (MH), and signs of systemic connective tissue involvement (SCTP). Material and methods. The study included 59 young men with MVP, MH, and SCTP. All participants underwent phenotypical and clinical examination, anthropometry, electrocard...

Aim. To assess systolic left ventricular (LV) function in patients with mitral valve prolapse (MVP) and different morphological MV disorders. Material and methods. The study included 233 patients (mean age 53,8±12,9 years), who underwent MV surgery due to MPV and severe mitral regurgitation (MR) at the V. A. Almazov Federal Centre of Heart, Blood,...

Aim. To assess the potential of plantography indices in the diagnostics of flat footedness, as a manifestation of Marfanoid habitus, systemic connective tissue involvement (SCTI), and cardiac involvement. Material and methods. In total, 50 young patients (mean age 22,5±2,1 years) were examined. In some participants, Marfanoid habitus and/or signs o...

Changes in activity of the components of TGF-β signaling pathway is associated with inherited disorders of connective tissue such as Marfan syndrome, Loeys-Dietz syndrome, etc. However, its impact on mitral valve prolapse (MVP) has not been completely studied. We examined 35 patients undergoing reconstructive surgery due MVP complicated by severe m...

Purpose: In some inherited connective tissue diseases with involving of the cardiovascular system, e.g. Marfan syndrome has been reported early impairment of LV function, which has been described as Marfan-related cardiomyopathy. Our aim was to evaluate the LV function in young adults with mitral valve prolapse (MVP) without significant mitral regu...

The paper attempts to revise the working classification of minor heart anomalies (MHA). It is proposed to exclude some syndromes and abnormalities which clearly have an independent clinical significance. The need for exclusion of prevalent normal variants, as well as anatomical and physiological characteristics of child’s heart, is justified. The i...

Purpose: Degenerative mitral valve disease (DMVD) often results in severe mitral valve regurgitation due to leaflet prolapse or chordal rupture. To select the most appropriate repair technique a complete understanding of the underlying degenerative etiology (Barlow's disease, BD or fibroelastic deficiency, FED) is mandatory. The purpose of this stu...

Purpose: Changes in activity of the components of TGF-β signaling pathway is associated with inherited disorders of connective tissue such as Marfan syndrome, Loeys-Dietz syndrome, etc. However, its impact on mitral valve prolapse (MVP) has not been complete studied. Our aim was to estimate the relationship between the concentration of TGF-β1/2 and...

Purpose: Epidemiology of mitral valve prolapse (MVP) has been described in different ethnic groups with controversy data on prevalence depending on age, sex, echocardiographic criteria and ethnicity itself. We studied prevalence of MVP in russian population using a large echocardiographic database.Methods: We retrospectively analyzed 16.185 echocar...

objective: To investigate the pathogenetic mechanisms of development of ascending aorta aneurysm (AAA) on the example of a cohort study patients of Almazov Federal Heart, Blood and Endocrinology Centre. Design and methods: A retrospective analysis was performed of the standard transthoracic echocardiography of 6345 pts. During the operation in 32 p...

Introduction: The mobility is one of major parameters of physical functioning. The new approaches to estimation of mobility are offered in International Classification of Functioning, Disability and Health (ICF), which was officially endorsed by all 191 WHO Member States in the 54 World Health Assembly on 2001. In clinical settings ICF is used for...

Objective: To assess the relation of cardiovascular risk factors to cardiovascular remodeling in the subjects with metabolic syndrome (MS). Design and Methods: 1600 mentally working subjects were screened for MS. 377 (127 males and 250 females) subjects who had at least one component of MS, no established cardiovascular events passed echocardiograp...

The paper presents echocardiography examination results in healthy young people. The prevalence of classic and non-classic mitral valve prolapse (MVP) is assessed. The prevalence of primary myxomatous MVP is independent of gender or age, while non-classic MVP is more prevalent in younger individuals. The mitral valve morphology is compared to that...

Purpose: Normal values for longitudinal segmental strain and strain rate (SR) have been reported in some population studies. However, are limited data about distribution of segmental radial and circumferential deformation in healthy young population. Methods: We studied 198 young subjects (mean age 20,1±1,6, 72% female) from PEPLICA study (prevalen...

One of the approaches to treatment of the aortic root aneurysms combined with aortic insufficiency is valve-sparing aortic root replacement. An analysis of immediate and long-term results dealt with 19 operations of replacement of the ascending parts of the aorta with sparing the aortic valve by the David I modified technique. The results demonstra...

The paper discusses the modern state of the mitral valve prolapse (MVP) problem. Controversial and unresolved issues on terminology, diagnostics, and tactics for different MVP variants are considered on the basis of the "Heritable disorders of connective tissue" recommendations (2009) by Expert Committee, the Society of Cardiology of the Russian Fe...