Cardiomyopathy WHO classification. Dilated cardiomyopathy. Ischemic conditions of the myocardium

“... any classification is incomplete and acts as a bridge between complete ignorance and absolute understanding...” (Goodwin J.F. The frontiers of cardiomyopathy // Brit. Heart. J. - 1982. - Vol. 48. - P.1-18.)

"Cardiomyopathy" (CM) translated from Greek (kardia - heart; mys, myos - muscle; pathos - suffering, disease) means "disease of the heart muscle." This term was first proposed by W. Bridgen in 1957 and was used to refer to myocardial diseases of unknown etiology, characterized by the appearance of cardiomegaly, ECG changes and a progressive course with the development of circulatory failure and an unfavorable prognosis for life. The same interpretation of the ILC was adhered to by J.F. Goodwin, which in the period 1961–1982. conducted a number of fundamental studies on this problem. In 1973, he proposed the following definition of CMP: “acute, subacute or chronic damage to the cardiac muscle of unknown or unclear etiology, often involving the endocardium or pericardium, and not due to structural deformation of the heart, hypertension (systemic or pulmonary) or coronary atheromatosis.” It was J.F. Goodwin first identified three groups of CMP: congestive (dilated - DCM), hypertrophic (HCM) and restrictive (RCMP).

The next step was a meeting of a special group of experts of the WHO, the International Society and the Federation of Cardiology (WHO/IFC) in 1980. In its report, the WHO/IFC defined CMP as “a disease of the heart muscle of unknown etiology.” At the same time, three groups of myocardial diseases were identified: unknown etiology (KMP), specific (of known etiology or associated with lesions of other organs and systems) and unspecified (cannot be attributed to any of the above groups). According to the 1980 WHO/IFC report, the term “cardiomyopathy” should have been used only in relation to myocardial diseases of unknown etiology and not used in relation to diseases of known etiology. This classification reflected the actual state of knowledge at that time: the etiology of the vast majority of CMPs was unknown and, therefore, they were considered idiopathic.

In 1995, a WHO/IFC expert working group revised the nomenclature and classification issues and proposed calling CMP “myocardial diseases that are associated with cardiac dysfunction.” At the same time, it was recommended to use the term “specific cardiomyopathy” to refer to myocardial lesions of known etiology or that are a manifestation of systemic diseases. This was a serious step forward. First, the term “cardiomyopathy” itself was clarified. Secondly, a number of new nosological units were introduced into the classification. For the first time, arrhythmogenic right ventricular cardiomyopathy was identified. The subsection of “unclassified” CMP was significantly expanded, which included fibroelastosis, noncompacted myocardium, systolic dysfunction with minimal dilatation, and mitochondrial involvement. The group of “specific” CMP was clarified and expanded, which included ischemic, valvular, hypertensive, peripartum CMP, etc. The change in terminology and clarification of the classification became possible thanks to scientific advances in the field of studying the etiology and pathogenesis of CMP. In particular, the role of viral infection in the origin of not only myocarditis, but also idiopathic dilated cardiomyopathy has been more clearly defined. Much data has emerged on the pathogenetic role of genetic factors in the development of CMP. As a result, the lines between idiopathic and specific CMPs began to blur.

Over the past 20 years, tremendous advances have been made in understanding the mechanisms of myocardial dysfunction and damage. A large number of clinical and population studies have been conducted, invasive and non-invasive research methods have been introduced and improved (echocardiography, Doppler echocardiography, magnetic resonance and computed tomography, endomyocardial biopsy, radioisotope research methods, etc.), and new histological data have been obtained. The use of molecular biology and genetics methods played an important role in clarifying the pathogenesis of CMP. These methods have contributed to a deeper understanding of the molecular basis of pathological processes in the myocardium. With the in-depth study of CMP, not only new diseases were identified, but also a number of difficulties appeared in determining their “class”. Increasingly, early and atypical manifestations of the disease, the development of a pathological process with minimal classical manifestations, and unusual forms that do not belong to any of the generally accepted categories of diseases have begun to be identified. As genetic research has advanced, medical science has faced a number of challenges. Firstly, the existence of a whole group of ILCs that are inherited was finally proven. Secondly, the question arose about the lack of a clear division between the concepts of “norm” and “not normal” in people with genetic disorders. Third, as a wide range of mutations leading to the development of CMP have been identified, a serious problem with the “overlap” of phenotypes has arisen. At the beginning, it was generally accepted that mutations in one gene lead to the development of one disease. Today the genetic formula has expanded significantly. It is already known that mutations of one gene can cause the development of many diseases with different phenotypic manifestations. Moreover, it has been proven that the development of one disease can be caused by mutations in several genes. Fourthly, many questions have arisen due to the lack of correlation between macro- and microscopic features in a number of diseases. An example is one of the familial forms of HCM with a morphological picture characteristic of this disease and the absence of significant hypertrophy of the walls.

In recent years, publications have increasingly begun to appear in which not only the need to revise the existing classification is discussed, but also new variants are proposed. In particular, in 2004, work was published by a group of Italian researchers, which expressed the opinion that the term “cardiac dysfunction” should imply not only decreased contractility and impaired diastolic function, but also rhythm disturbances, conduction system, and a state of increased arrhythmogenicity (enhanced arrhythmogenicity). In particular, this work raised the question of whether CMP should be considered myocardial dysfunction without visible structural changes, leading to the development of life-threatening cardiac arrhythmias and a high risk of sudden cardiac death? The authors discussed the inclusion of a number of pathologies in the classification of CMP in which genetic defects lead to disruption of ion channels and the risk of developing “electrical paralysis” of the heart. The same work presents a genomic or “molecular” classification of hereditary CMPs. Three groups of diseases have been proposed:

1. cytoskeletal CMP (or “cytoskeletopathies”): DCM, arrhythmogenic right ventricular dysplasia (ARVD) and CMP with skin manifestations (Cardiocutaneous syndromes) (E. Norgett et al., 2000);
2. sarcomeric CMP (or “sarcomeropathies”): HCM, RCM;
3. Ion channel CMP (or “channelopathies”): long and short QT interval syndromes, Brugada syndrome, catecholaminergic polymorphic VT.

In 2006, a new American Heart Association (AHA) classification of CMP was published. It proposed a new definition of CMP as "a heterogeneous group of myocardial diseases associated with mechanical and/or electrical dysfunction, which usually (but not without exception) manifest as inappropriate hypertrophy or dilatation and arise from a variety of causes, often genetic . CMP is limited to the heart or is part of generalized systemic disorders, always leading to cardiovascular death or progression of heart failure...” This classification highlighted:

• Primary cardiomyopathy: isolated (or prevalent) myocardial damage.
• Secondary cardiomyopathy: myocardial damage is part of generalized systemic (multiorgan) diseases.

Among the primary CMPs the following are identified:

• Genetic:
  • HCM;
  • ARVD;
  • non-compact myocardium of the left ventricle;
  • disorders of glycogen storage;
  • PRKAG2 (protein kinase, AMP-activated, gamma 2 non-catalytic subunit);
  • Danon disease;
  • conduction defects;
  • mitochondrial myopathies;
  • ion channel disorders (long QT syndrome (LQTS); Brugada syndrome; short QT syndrome (SQTS); Lenegre syndrome; catecholaminergic polymorphic ventricular tachycardia (CPVT); unexplained sudden nocturnal death syndrome (Asian SUNDS)).
• Mixed:
  • DCM and RCM.
• Purchased:
  • inflammatory (myocarditis);
  • stress-induced (takōtsubo);
  • peripartum;
  • tachycardia-induced;
  • in children born to mothers with insulin-dependent diabetes mellitus.

At first glance, the classification may seem complex and confusing. However, a more detailed examination shows that it is based on two simple principles. Firstly, as in the previous classification, the division according to the “cause-and-effect” principle is preserved: primary and secondary ILCs are distinguished. Secondly, the principle of division is used depending on the possibility of inheritance. Primary CMPs are divided into three groups: hereditary (familial/genetic), non-hereditary (acquired) and mixed CMPs. “Mixed CMP” refers to a group of diseases that can be caused by both genetic defects and develop as a result of the influence of various factors.

What's new in this classification? Its main fundamental differences from previous classifications are:

• new definition of ILC;
• lack of a principle of primary grouping depending on anatomical features;
• for the first time in the official classification, the principle of dividing the IMC depending on the possibility of inheritance was applied;
• new types of ILC have been identified.

Let's look at these differences in more detail.

First, the current classification of the American Heart Association (AHA) recognizes that CMP is a “heterogeneous group” of diseases. In addition, the definition for the first time stated that CMP may be based not only on “mechanical” but also on “electrical” dysfunction. In this regard, “ion channel disorders” or “channelopathies” have been introduced into the group of genetic CMPs. It is assumed that since mutations in ion channel genes are responsible for disruption of the biophysical properties and structure of proteins, i.e. for changes in the structure of surfaces and the architecture of ion channels, then, therefore, we can say that “channelopathies” are a pathology of cardiomyocytes, that is, a disease of the myocardium, and they can be considered CMP.

Secondly, there is no “general” identification of forms of CMP depending on the phenotype, or in other words, on anatomical features. In the new AAS classification, DCM, HCM, RCM and ARVC are actually the third subclass of “primary” CMP. The new classification also does not include “idiopathic,” “specific,” or “unclassified” CMPs. Some of the CMPs that were previously classified in these categories ("non-compact myocardium", mitochondrial CMP, inflammatory CMP, peripartum CMP) are included in the main groups of the modern classification of CMP. Others - fibroelastosis, ischemic, valvular, hypertensive CMP - are not classified as CMP at all.

Thirdly (and this is very important), in the new classification of AAS, in contrast to previous official classifications, for the first time the principle of dividing IMC depending on the possibility of inheritance is used. What does this mean? For the first time, the presence of certain types of ILC, which can be inherited, has been officially recognized. It would seem, what's new in this? The works of J. Towbin et al. are well known. (1994, 2000), P.J. Keeling et al. (1995), K. Bowles et al. (1996), L. Mestroni (1997, 1999). The scientific literature has been discussing the “family” ILC for several years. However, this is the first time such a division has been used in the official classification of the Society of Cardiologists.

Fourthly, the group of acquired ILCs was clarified. For the first time, such forms as tachycardia-induced, stress-induced (takоtsubo) and CMP in children whose mothers suffer from insulin-dependent diabetes mellitus have been identified.

In 2008, a new classification of the European Society of Cardiology (ESC) was published. This classification, as its authors indicate, was created not only to clarify the concept and update the division of CMP into groups, but also for widespread use in everyday clinical practice. Currently, in most clinics in the world, it is not possible to conduct extensive research to identify genetic mutations before the onset of clinical symptoms or before the incidental detection of myocardial pathology. Moreover, the presence of an established genetic defect in a family is not always accompanied by clinical and/or morphological manifestations. In addition, treatment of such patients is extremely rarely started before the diagnosis of CMP is established. Therefore, the EOC classification is more clinically oriented and is based on the division of CMP depending on the morphological and functional changes in the myocardium of the ventricles of the heart.

The EOC defines the concept of ILC somewhat differently than the AAS. According to the ESC, CMP is “a pathology of the myocardium in which its structural or functional disorders occur that are not caused by coronary heart disease, hypertension, valvular defects and congenital heart diseases...” CMP are grouped depending on the morphological or functional phenotype:

• HCM.
• DCM.
• APZhD.
• RCMP.

Unclassified: non-compact myocardium, Takotsubo cardiomyopathy.

All ILC phenotypes, in turn, are divided into:

• Family/family (genetic):
  • unidentified gene defect;
  • subtype of the disease.
• Non-familial/non-family (non-genetic):
  • idiopathic;
  • subtype of the disease.

The division of CMP into familial and non-familial is intended to increase physicians' awareness of the genetic determinants of CMP and direct them to conduct specific diagnostic tests, including the search for specific mutations in appropriate cases.

The diagnosis of DCM should be made in cases of dilatation and impaired systolic function of the left ventricle in the absence of causes (coronary heart disease, valvular pathology, hypertension) leading to their development. The DCM phenotype can develop due to mutations in various genes encoding cytoskeletal proteins, sarcomeric proteins, Z-disks, nuclear membranes, defects in the X chromosome, etc. Manifestations of DCM may be present in mitochondrial cytopathies, metabolic disorders (hemochromatosis), deficiency conditions, endocrine diseases, when using cardiotoxic medications, in the late stages of inflammatory processes in the myocardium. A separate form of DCM with moderate ventricular dilatation has been identified: mildly dilated congestive cardiomyopathy. This form is diagnosed in patients with heart failure with severe systolic dysfunction in the absence of significant dilatation (an increase of only 10-15% compared with normal) or restrictive hemodynamics. DCM also includes peripartum cardiomyopathy, which develops in the last month of pregnancy or within 5 months after birth.

Previously, HCM was defined as the development of myocardial hypertrophy not associated with hemodynamic stress and systemic diseases such as amyloidosis or glycogen storage disorders. It was believed that it was necessary to differentiate true hypertrophy of cardiomyocytes from that caused by interstitial infiltration or intracellular accumulation of metabolic substrates. The modern EOC classification proposes a more simplified definition of HCM: “the presence of a thickened wall or an increase in myocardial mass in the absence of factors contributing to their development (hypertension, valvular defects).” This allows us to interpret the term “HCM” somewhat more broadly and not be limited only to a certain phenotype with a single etiology (for example, pathology of sarcomeric proteins).

In the new classification, RCM is defined as a physiological state of the myocardium with normal or reduced volumes (diastolic and systolic) of the cavity of the heart ventricle (one or two) and normal thickness of its (their) walls. It is necessary to distinguish primary RCMP, or idiopathic, from secondary - developed as a result of systemic diseases such as amyloidosis, sarcoidosis, carcinoid disease, scleroderma, anthracycline CM, fibroelastosis, hypereosinophilia syndrome, endomyocardial fibrosis.

The EOC classification is indeed more simplified and closer to clinical practice than the one proposed by the AAS. It provides a greater degree of freedom for making a clinical diagnosis of CMP. However, there is a certain disadvantage to this. For example, the possibility of a broader interpretation of the diagnosis of HCM or a subtype of DCM. In the latter case, the ESC classification suggests considering DCM as sporadic (non-familial, non-genetic) in the absence of the disease in other family members. It is proposed to divide sporadic DCM into “idiopathic” and “acquired”. At the same time, it is indicated that acquired cardiomyopathy are those in which ventricular dysfunction “...is more likely a complication of the disease than its direct manifestation.” However, they miss the fact that, for example, with mutations in mitochondrial RNA, the development of a CMP phenotype is possible, which should be considered both “acquired” and “genetic”. However, these mutations are not necessarily transmitted to subsequent generations.

In conclusion, I would like to note that the emergence of new classifications of AAS and EOC indicates the accumulation of a large amount of new information about the etiology of CMP and a deeper understanding of the pathogenetic mechanisms of this group of diseases. At the same time, these classifications should be regarded only as the next stage, which brings us closer to a complete understanding of the pathological process. The revision of definitions and classification by international societies makes it necessary to make changes to the domestic ILC classification. In this regard, below are drafts of new classifications of cardiomyopathy and myocarditis, which are proposed for use in Ukraine. The projects take into account the changes proposed by the EOC and the AAS.

Literature

1.Bowles K., Gajarski R., Porter P. et al. Gene mapping of familial autosomal dominant dilated cardiomyopathy to chromosome 10q21-23 // J. Clin. Invest. – 1996. – Vol. 98. – P. 1355-1360.

2.Bridgen W. Uncommon myocardial diseases – the noncoronary cardiomyopathies // Lancet. – 1957. – Vol. 2. – P. 1243-1249.

3.Cooper L.T., Baughman K.L., Feldman A.M. et al. The Role of endomyocardial biopsy in the management of cardiovascular disease: A scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology // J. Amer. Coll. Cardiology. – 2007. – Vol. 50. – P. 1914-1931

4.Elliott P., Andersson B., Arbustini E. et al. Classification of the cardiomyopathies: a position statement from the European society of cardiology working group on myocardial and pericardial diseases // Eur. Heart. J. – 2008. – Vol. 29, No. 2. – P. 270-276.

5.Keeling PJ., Gang G., Smith G. et al. Familial dilated cardiomyopathy in the United Kingdom // Brit. Heart. J. – 1995. – Vol. 73. – P. 417-421.

6.Maron B.J., Towbin J.A., Thiene G. et al. American Heart Association; Councilon Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; Council on Epidemiology and Prevention. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention // Circulation. – 2006. – Vol. 113. – P.1807-1816.

7.Mestroni L. Dilated cardiomyopathy: a genetic approach // Heart. – 1997. – Vol. 77. – P. 185-188.

8.Mestroni L., Maisch B., McKenna W. et al. Guidelines for the study of familial dilated cardiomyopathies // Eur. Heart J. – 1999. – Vol. 20. – P. 93-102.

9.Norgett E.E., Hatsell S.J., Carvajal-Huerta L. et al. Recessive mutation in desmoplakin disrupts desmoplakin-intermediate filament interactions and causes dilated cardiomyopathy, woolly hair and keratoderma // Hum. Mol. Genet. – 2000. – Vol. 9, No. 18. – P. 2761-2766.

10.Priori S., Napolitano C., Tiso N. et al. Mutations in the cardiacryanodine receptor gene (hRyR2) underlie catecholaminergic polymorphic ventricular tachicardia // Circulation. – 2001. – Vol. 103. – P. 196-200.

11.Priori S., Napolitano C. Genetic defects of cardiac ion channels. The hidden substrate for torsades de pointes // Cardiovasc. Drugs. Ther. – 2002. – Vol. 16. – P. 89-92.

12.Priori S., Schwartz P., Napolitano C. et al. Risk stratification in the long-QT syndrome // New Engl. J. Med. – 2003. – Vol. 348. – P.1866-1874.

13.Report of the WHO/ISFC task force on the definition and classification of cardiomyopathies // Brit. Heart J. – 1980. – Vol. 44. – P. 672-673.

14. Richardson D., McKenna W., Bristow M. et al. WHO/ISFC Task Force definition and classification of cardiomyopathies // Circulation. – 1996. – Vol. 93. – P. 841-842.

15. Thiene G., Corrado D., Basso C. Cardiomyopathies: is it time for a molecular classification? //Eur. Heart J. – 2004. - Vol. 25. – P. 1772-1775.

16.Towbin J., Hejtmancik F., Brink P. et al. X-linked dilated cardiomyopathy molecular genetic evidence of linkage to the Duchenne muscular dystrophy (dystrophin) gene at the Xp21 locus // Circulation. – 1993. – Vol. 87. – P. 1854-1865.

17.Towbin J.A., Bowles N.E. Genetic abnormalities responsible for dilated cardiomyopathy // Curr. Cardiol. Rep. – 2000. – Vol. 2. – P. 475-480.

V.N. Kovalenko, D.V. Ryabenko

National Scientific Center "Institute of Cardiology named after Academician N.D. Strazhesko" of the Academy of Medical Sciences of Ukraine, Kiev

Ukrainian Journal of Cardiology

Cardiomyopathy is pathological changes in myocardial tissue, leading to severe disturbances in the functioning of the heart muscle.

This disease has many forms, which differ in the causes of occurrence and the manifested clinical picture. In this regard, several types of classification of cardiomyopathy have been identified.

According to the mechanism of disease development

Most often, when diagnosing, they rely specifically on a classification based on the mechanism of development of the pathology. This is explained by the fact that this type of classification allows you to timely diagnose the form of the disease and plan the necessary treatment as early as possible.

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With this classification, 5 forms of cardiomyopathy are distinguished:

• specific;
• unclassifiable.

Dilatational

With dilated cardiomyopathy, excessive stretching of the heart walls occurs, which leads to expansion of the chamber cavities and increased pressure.

This form of cardiomyopathy is detected more often than others and at the primary stage of development is localized in one chamber. But it can cover both the atrium and the ventricles.

Damage to the ventricles is characterized by more severe symptoms and consequences.
Causes

Dysfunction of nervous system regulation	The transmission of signals from the heart to working cardiomyocytes is blocked. In this case, when the heart chambers are filled, the muscle excitation impulse is completely absent or has a weak force, which leads to stretching of the walls.
Decrease in the level of the main components responsible for myocardial contraction	Such a component is myofibrils. When their number decreases, the muscle tissue of the heart becomes less elastic and, as a result, it becomes overstretched. The main reasons for this phenomenon are most often ischemia and cardiosclerosis.
Blood electrolyte imbalance	Non-compliance with the standards for the amount of potassium, sodium and chlorine ions. These substances directly affect the contraction of the heart, and their absence leads to an increase in intracameral pressure and stretching of the walls.

As a rule, with excessive stretching of the myocardial walls, the cavity of the chambers increases, and, therefore, the volume of processed blood increases. In addition, the valve holes widen and a gap forms.

To pump an increased amount of blood, the heart begins to contract several times faster than normal. Loose closure of the valve leaflet provokes the return of blood from the ventricle to the atrium chamber.

Due to the heavy load, blood stagnation is possible not only in the cavity of the atrium or ventricle, but also in the systemic and pulmonary circulation.

Partial disruption of pumping function provokes the development of heart failure and is accompanied by characteristic manifestations.

To regulate the functioning of the heart and support the pumping function, the body includes some compensating mechanisms:

To diagnose dilated cardiomyopathy, several criteria are mainly used:

• an increase in the ventricular cavity to a maximum of 6 cm, which is recorded at the stage of muscular relaxation of the heart;
• reduction in expelled blood volume by 50% or more.

Hypertrophic

The hypertrophic form of cardiomyopathy is characterized by an increase in the density and thickness of the heart walls by 1.5 cm or more, without changes or with a partial decrease in the size of the cavity.

The localization area is the ventricles or their septum. It has been noted that the right ventricle is less susceptible to this disease than the left. And thickening of the septum is observed only in isolated cases.

There are two types of hypertrophic cardiomyopathy:

Studies have shown that the hypertrophic form of cardiomyopathy is provoked by the following factors:

• high level of insulin production;
• exposure to catecholamines, leading to disruption of neurotransmitter metabolism;
• hypertension;
• thyroid dysfunction;
• various processes accompanied by genomic mutation;
• wrong lifestyle.

When the walls of the ventricles thicken, a disorderly proliferation of heart muscle fibers is observed and the size of the ventricular cavity decreases. This leads to an increase in the pressure of the processed blood, and further to stretching of the walls of the heart.

The thickening covers not only the ventricle, but also the walls of the coronary artery, which leads to a decrease in its lumen. Taken together, all aspects lead to congestion in the pulmonary circulation and cause a number of serious complications.

Restrictive

The restrictive form of cardiomyopathy is characterized by a decrease in the elasticity of the heart muscle, in which the cavities cannot expand appropriately and process the standard volume of blood flow. It can affect either one or both ventricles.

Causes:

1. Massive proliferation of fibrous tissue.
2. An accumulation of substances that are not part of the heart apparatus.

The proliferation of fibrous tissue leads to compaction and thickening of the heart muscle, which leads to a decrease in the cavity and an increase in the pressure of the passing blood flow. Because of this, there is stagnation in the atrium and, as a result, their expansion occurs.

The consequences of the development of this type of cardiomyopathy can be the following manifestations:

• fibrosis of the idiopathic type, which acts as the primary manifestation of the restrictive form of cardiomyopathy;
• Loeffler's fibroplastic endocarditis.

The listed forms of the disease are basic and generally recognized. The remaining forms considered do not always correspond to other classifications of cardiomyopathy.

Specific

Specific forms of cardiomyopathy include secondary lesions of the heart muscle. They often have similar symptoms to the three main forms of cardiomyopathy. But there is a significant difference - specific forms are a complication, and not the main pathology.

According to the causes, the following types of disease are distinguished:

• allergic;
• inflammatory;
• ischemic.

The peculiarity of this form of cardiomyopathy is that the symptoms of each type differ little from each other.

Unclassifiable

An unclassified form of cardiomyopathy is a pathology that includes symptoms of several forms of the disease.

In this case, a combination of atrial dilatation and ventricular hypertrophy is possible.

The causes of the unclassifiable form can be quite varied, and refers to three main forms.

According to WHO (1995)

After a detailed study of cardiomyopathy in 1995, a different WHO classification of cardiomyopathy was formed, which identifies the following forms of pathology, divided into groups:

According to Goodwin

In 1966, the English scientist Charles Goodwin created his own classification, which is still used by the majority of practicing cardiologists.

Keywords

DILATED CARDIOMYOPATHY/ CLASSIFICATION / DIAGNOSTICS / CLINICAL CASE STUDY

annotation scientific article on clinical medicine, author of the scientific work - Mukhametgalieva Gulnaz Munirovna, Oshchepkova Olga Borisovna, Tsibulkin Nikolay Anatolyevich, Tukhvatullina Galina Vladimirovna, Mikhoparova Olga Yurievna

The goal is to analyze modern ideas about dilated cardiomyopathy in the daily clinical practice of a cardiologist and present clinical example. Material and methods. A review of scientific medical literature on the topic was conducted dilated cardiomyopathy. The results of observation and clinical examination of a patient diagnosed with dilated cardiomyopathy. Results and its discussion. Dilated cardiomyopathy is a myocardial disease of various etiologies, characterized by a pronounced dilation of the left ventricular cavity and a decrease in global contractility in the absence of an underlying cardiac disease. Such patients are characterized by the development of progressive heart failure, cardiac arrhythmia and conduction disturbances, thromboembolism, and sudden death. The criterion for the disease is a decrease in the ejection fraction of the left ventricle and a pronounced increase in the size of its cavity. Patients with dilated cardiomyopathy occupy from a quarter to half of all cases of cardiomyopathies. In the genesis of the disease, the interaction of factors such as genetic predisposition, exposure to exogenous factors, and autoimmune disorders is important. In most cases, the cause of the disease is the structural and functional deficiency of contractile or structural proteins in cardiomyocytes. Of the patients admitted to the hospital with symptoms of progressive chronic heart failure, a little more than half have an ischemic origin of the disease, while the diagnosis of the idiopathic form requires additional research. Conclusions. Modern idea of dilated cardiomyopathy includes determining the primary causes of characteristic changes in the heart due to the underlying disease, identifying specific cardiomyopathies, as well as assessing the role of hereditary factors in the genesis of this disease.

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DILATED CARDIOMYOPATHY:MODERN PERCEPTION AND CLINICAL CASE

Aim. The aim of the study was to analyze the modern perception of dilated cardiomyopathy in everyday clinical practice of a cardiologist and to provide a clinical example. Materials and methods. Review of scientific medical literature on the topic of dilated cardiomyopathy was conducted. The results of observation and clinical examination of a patient with a diagnosis of dilated cardiomyopathy are presented. Results and discussion. Dilated cardiomyopathy is a disease of the myocardium of multiple origins, characterized by a marked expansion of the left ventricular cavity and a decrease in global contractility in the absence of a major cardiac disease. Such patients are characterized by progressive heart failure development, heart rhythm and conduction disorders, thromboembolism and sudden death. The criterion of the disease is the decrease in the left ventricle ejection fracture and a pronounced increase in its cavity size. Patients with dilated cardiomyopathy occupy from a quarter to half among all cases of cardiomyopathies. The interaction of such factors as genetic predisposition, exposure to exogenous factors and autoimmune disorders is important for the initiation of the disease. In most cases, the cause of the disease is the structural and functional inadequacy of contractile or structural proteins in cardiomyocytes. Just over half of the patients admitted to the hospital with the symptoms of progressive chronic heart failure have ischemic origin of the disease, while the diagnosis of the idiopathic type requires additional investigation. Conclusion. The modern understanding of dilated cardiomyopathy includes the definition of primary causes of the characteristic changes in the heart due to the underlying disease, identification of specific cardiomyopathies, as well as assessment of the role of hereditary factor in the initiation of the disease.

Text of scientific work on the topic “Dilated cardiomyopathy: modern concepts and an example of clinical course”

PRACTICAL EXPERIENCE

© PM. Mukhametgalieva, O.B. Oshchepkova, N.A. Tsibulkin, G.V. Tukhvatullina, O.Yu. Mikhoparova, 2018

UDC 616.127 DOI: 10.20969/VSKM.2018.11(4).113-119

DILATED CARDIOMYOPATHY:

MODERN CONCEPTS AND AN EXAMPLE OF CLINICAL COURSE

MUKHAMETGALIEVA GULNAZ MUNIROVNA, cardiologist of the Department of Cardiology, Clinical Hospital of the Medical Unit of the Ministry of Internal Affairs of the Russian Federation for the Republic of Tatarstan, Russia, 420059, Kazan, Orenburgsky tract, 132

OSHCHEPKOVA OLGA BORISOVNA, head. Department of Cardiology, Clinical Hospital of the Medical Emergency Department of the Ministry of Internal Affairs of the Russian Federation for the Republic of Tatarstan, Russia, 420059, Kazan, Orenburgsky tract, 132

TSIBULKIN NIKOLAY ANATOLIEVICH, Ph.D. honey. Sciences, Associate Professor of the Department of Cardiology, X-ray Endovascular and Cardiovascular Surgery of the KSMA - branch of the Federal State Budgetary Educational Institution of Further Professional Education RMANPO of the Ministry of Health of Russia, Russia, 420012, Kazan, st. Butlerova, 36, e-mail: [email protected]

TUKHVATULLINA GALINA VLADIMIROVNA, head. Clinical and diagnostic laboratory of the Federal Public Health Institution "Clinical Hospital of the Medical Unit of the Ministry of Internal Affairs of the Russian Federation for the Republic of Tatarstan", Russia, 420059, Kazan, Orenburgsky tract, 132

MIKHOPAROVA OLGA YURIEVNA, head. Department of Functional Diagnostics, Clinical Hospital of the Medical Emergency Department of the Ministry of Internal Affairs of the Russian Federation for the Republic of Tatarstan, Russia, 420059, Kazan, Orenburgsky tract, 132

Abstract. The goal is to analyze modern concepts of dilated cardiomyopathy in the daily clinical practice of a cardiologist and present a clinical example. Material and methods. A review of the scientific medical literature on the topic of dilated cardiomyopathy was conducted. The results of observation and clinical examination of a patient diagnosed with dilated cardiomyopathy are presented. Results and its discussion. Dilated cardiomyopathy is a myocardial disease of various etiologies, characterized by pronounced dilation of the left ventricular cavity and a decrease in global contractility in the absence of an underlying cardiac disease. Such patients are characterized by the development of progressive heart failure, cardiac arrhythmia and conduction disturbances, thromboembolism, and sudden death. The criterion for the disease is a decrease in the ejection fraction of the left ventricle and a pronounced increase in the size of its cavity. Patients with dilated cardiomyopathy account for between a quarter and half of all cases of cardiomyopathies. In the genesis of the disease, the interaction of factors such as genetic predisposition, exposure to exogenous factors, and autoimmune disorders is important. In most cases, the cause of the disease is the structural and functional deficiency of contractile or structural proteins in cardiomyocytes. Of the patients admitted to the hospital with symptoms of progressive chronic heart failure, a little more than half have an ischemic origin of the disease, while the diagnosis of the idiopathic form requires additional research. Conclusions. The modern understanding of dilated cardiomyopathy includes determining the primary causes of characteristic heart changes due to the underlying disease, identifying specific cardiomyopathies, as well as assessing the role of hereditary factors in the genesis of this disease. Key words: dilated cardiomyopathy, classification, diagnosis, clinical example. For reference: Dilated cardiomyopathy: modern concepts and an example of clinical course / G.M. Mukhametgalieva, O.B. Oshchepkova, N.A. Tsibulkin [et al.] // Bulletin of modern clinical medicine. -2018. - T. 11, issue. 4. - P.113-119. DOI: 10.20969/VSKM.2018.11(4).113-119.

DILATED CARDIOMYOPATHY:

MODERN PERCEPTION AND CLINICAL CASE

MUKHAMETGALIEVA GULNAZ M., cardiologist of the Department of cardiology of Clinical Hospital of the Ministry of Internal Affairs of Russia for the Republic of Tatarsan, Russia, 420059, Kazan, Orenburgskiy tract str., 132 OSHCHEPKOVA OLGA B., Head of the Department of cardiology of Clinical Hospital of the Ministry of Internal Affairs of Russia for the Republic of Tatarsan, Russia, 420059, Kazan, Orenburgskiy tract str., 132 TSYBULKIN NIKOLAY A., C. Med. Sci., associate professor of the Department of cardiology, x-ray endovascular and cardiovascular surgery of Kazan State Medical Academy - the branch of Russian Medical Academy of Postgraduate Education, Russia, 420012, Kazan, Butlerovstr., 36, e-mail: [email protected]

TUKHVATULLINA GALINA V., Head of laboratory of clinical diagnostics of Clinical Hospital of the Ministry of Internal Affairs of Russia for the Republic of Tatarsan, Russia, 420059, Kazan, Orenburgskiy tract str., 132 MIKHOPAROVA OLGA YU., Head of the Department of functional diagnostic of Clinical Hospital of the Ministry of Internal Affairs of Russia for the Republic of Tatarsan, Russia, 420059, Kazan, Orenburgskiy tract str., 132

Abstract. Aim. The aim of the study was to analyze the modern perception of dilated cardiomyopathy in everyday clinical practice of a cardiologist and to provide a clinical example. Materials and methods. Review of scientific medical literature on the topic of dilated cardiomyopathy was conducted. The results of observation and clinical examination of a patient with a diagnosis of dilated cardiomyopathy are presented. Results and discussion. Dilated cardiomyopathy is a disease of the myocardium of multiple origins, characterized by a marked expansion of the left ventricular cavity

and a decrease in global contractility in the absence of a major cardiac disease. Such patients are characterized by progressive heart failure development, heart rhythm and conduction disorders, thromboembolism and sudden death. The criterion of the disease is the decrease in the left ventricle ejection fracture and a pronounced increase in its cavity size. Patients with dilated cardiomyopathy occupy from a quarter to half among all cases of cardiomyopathies. The interaction of such factors as genetic predisposition, exposure to exogenous factors and autoimmune disorders is important for the initiation of the disease. In most cases, the cause of the disease is the structural and functional inadequacy of contractile or structural proteins in cardiomyocytes. Just over half of the patients admitted to the hospital with the symptoms of progressive chronic heart failure have ischemic origin of the disease, while the diagnosis of the idiopathic type requires additional investigation. Conclusion. The modern understanding of dilated cardiomyopathy includes the definition of primary causes of the characteristic changes in the heart due to the underlying disease, identification of specific cardiomyopathies, as well as assessment of the role of hereditary factor in the initiation of the disease. Key words: dilated cardiomyopathy, classification, diagnostics, clinical case.

For reference: Muhametgalieva GM, Oschepkova OB, Tsybulkin NA, Tuhvatullina GV, Mihoparova OY. Dilated cardiomyopathy: modern perception and clinical case. The Bulletin of Contemporary Clinical Medicine. 2018; 11 (4): 113-119. DOI: 10.20969/VSKM.2018.11(4).113-119.

The diagnosis of dilated cardiomyopathy (DCM) defines myocardial diseases of various etiologies, characterized by pronounced dilation of the left ventricular (LV) cavity and a decrease in global contractility in the absence of hypertension, coronary heart disease (CHD) and heart defects. The development of progressive heart failure, cardiac arrhythmia and conduction disturbances, thromboembolism, and sudden death is characteristic. The criterion for the disease is a decrease in the left ventricular ejection fraction below 45% and the size of the left ventricular cavity in diastole more than 6 cm. Patients with DCM make up from 26 to 60% of all patients with cardiomyopathies (CM). In the genesis of DCM, the interaction of several factors is important: genetic predisposition to the occurrence of the disease; exposure to exogenous factors (viral infection, alcohol) and autoimmune disorders.

Currently, the concept of DCM also corresponds to severe decompensated heart failure of ischemic or non-ischemic origin. Ischemic CMP is classified as a form of CAD, and non-ischemic CMP is DCM itself. If the etiology is uncertain, DCM is defined as idiopathic. In most cases, the cause of this disease is the structural and functional inferiority of contractile or structural proteins in cardiomyocytes. Of the patients admitted to the hospital with symptoms of progressive chronic heart failure (CHF), just over half have ischemic cardiomyopathy. At the same time, the proportion of idiopathic DCM in this group cannot be reliably determined without additional studies.

The classification of ILC has undergone more than one change; Currently, two of them are used in parallel. The classification of the European Society of Cardiology distinguishes dilated, hypertrophic, restrictive, unclassified and arrhythmogenic right ventricular CMP, each of which is divided into familial (inherited) and non-familial (sporadic). The World Heart Federation classification, also designated MOGE(S), includes a division according to morphological characteristics (M), characteristics of organ damage (O), genetic

European determination of etiological factors (E) and state of function.

Depending on the definition used in the epidemiological assessment, idiopathic DCM occurs in 4 to 8 cases per 100,000 adults per year. However, since some patients are asymptomatic, the prevalence of this disease is likely higher. The nature of the course of DCM is also not clearly defined due to the fact that the onset of the disease, the clinical manifestation of which is chronic heart failure, can be asymptomatic for a long time. In addition, this diagnosis combines cases with different etiologies and, accordingly, with different prognoses. About a quarter of cases of DCM have a good prognosis and restoration of LV contractility. The nature of the treatment also plays a significant role. The use of drugs that increase survival in CHF determines a significant improvement in prognosis in most patients with DCM.

Men get sick 2-3 times more often than women, especially at the age of 30-50 years. Significant racial differences have been identified in the course of this disease. People of black race have a threefold higher risk of developing DCM and a twofold higher risk of mortality from this disease. Women are less likely to suffer from DCM, however, once the disease has occurred, no significant gender differences in the course and prognosis of DCM have been identified to date. At the same time, a number of factors are predictors of an unfavorable prognosis. These include: parallel involvement of both ventricles, relative mitral valve insufficiency, impaired intraventricular conduction (left bundle branch block), CHF stage MB and higher, increased levels of brain natriuretic peptide, signs of myocardial damage according to laboratory data (troponins) and biopsy results. Low exercise tolerance, concomitant parenchymal organ failure (renal, hepatic), pulmonary hypertension of any origin and advanced age also worsen the prognosis.

Principles of diagnosing DCM. Diagnosis of DCM begins with clinical identification of patients

ents with signs of CHF and expansion of the borders of the heart to the left. Next, the patient needs to undergo transthoracic echocardiography (EchoCG) according to a standard or screening protocol for possible detection of cardiac diseases, assessment of the degree of chamber dilatation and the state of the integral contractile function of the LV. Registration of an ECG is necessary, as it can help clarify the etiology of dilatation of the heart chambers and the origin of CHF. X-ray of the chest organs in a direct projection in such patients is an important universal diagnostic screening method. Laboratory tests include general and biochemical blood tests to assess kidney and liver function, electrolyte balance, hemoglobin HbA1c levels, assessment of iron metabolism and thyroid function (possibly in combination with ultrasound data), and a test for HIV infection. An important role is played by collecting anamnesis, which should contain information about the possible causes of DCM (hypertension, nutritional factor). Heritability assessment may require collecting information from the patient's relatives, as data from at least three generations in the family is required. Data are needed on the amount and composition of long-term drug therapy taken by the patient, with an emphasis on drugs with cardiotoxic effects. A history of infectious diseases and an obstetric and gynecological history for women may also contain relevant information.

During the diagnostic examination, special attention should be paid to identifying signs of coronary atherosclerosis and clinical symptoms of coronary heart disease (CHD). Stress and drug-induced functional tests, stress echo, Holter ECG monitoring, monitoring the clinical condition and patient complaints are an effective means of non-invasive diagnosis of coronary artery disease. Cardiovascular risk factors should also be taken into account, as they are independent predictors of prognosis in any patient. Confirmation of the presence of clinically significant manifestations of atherosclerotic lesions of the coronary arteries in the patient gives grounds to consider this case as ischemic cardiomyopathy. In middle-aged patients with cardiovascular risk factors, active detection of coronary artery disease using exercise testing may be justified. The question of the possible combination of genetically determined, both sporadic and familial CMP with symptoms of IHD; the interpretation of such cases and the tactics for their management remain open.

Excluding the ischemic origin of DCM when signs of heart failure and cardiac dysfunction persist or worsen, despite treatment, requires further in-depth diagnosis. Magnetic resonance or x-ray computed tomography may be useful in identifying myocardial lesions in "storage diseases" such as amyloidosis, hemochromatosis, mucopolysaccharidosis,

as well as for the diagnosis of sarcoidosis and tumors of cardiac localization. In the latter case, echocardiography may not provide the necessary diagnostic data for infiltrative tumor growth. One of the possible alternatives in differential diagnosis is severe myocarditis. Confirmation or exclusion of this diagnosis is possible by performing a biopsy.

Myocardial biopsy is an invasive method and should be used only if its results can determine the specific direction of treatment and tactics for further management of the patient. Such cases include “storage diseases”, eosinophilic myocarditis, sarcoidosis. Laboratory studies should also be aimed at identifying specific causes of CMP. Research for rheumatological diseases and collagenoses, as well as laboratory screening for possible use of alcohol or toxic substances, may be informative. Alcoholic heart disease should be one of the main diseases considered as a cause of DCM in patients with concomitant parenchymal liver pathology and a corresponding life history.

Consultation with an endocrinologist and appropriate laboratory tests help determine the causes of cardiac dysfunction in thyrotoxicosis and other diseases of a hormonal nature. Identification of infectious and immune inflammatory mechanisms of myocardial damage by serological methods can also clarify the genesis of characteristic changes in the heart. In the absence of signs of underlying cardiovascular disease and evidence of secondary cardiac damage and specific cardiomyopathy, the idiopathic form of DCM can be diagnosed. At the same time, the results of such an extensive clinical and instrumental examination cannot be ignored and must be reflected in the diagnosis. This opportunity is provided by the new classification of cardiomyopathies MOGE(S). Each of its elements listed above contains several (up to ten or more) assessment options, which are determined by the results of the survey. Thus, most clinical and diagnostic indicators are included in the classification characteristics of CMP. In addition, such an expanded classification allows you to quickly and accurately determine a general understanding of the patient, his condition and prognosis, and also indicates possible options for management tactics.

Clinical example. Patient Z., 49 years old, was admitted to the cardiology department in March 2016 with complaints of inspiratory shortness of breath at rest, attacks of suffocation in a horizontal position, fatigue, discomfort in the heart area with minor physical activity, and swelling of the legs. From the anamnesis: no heredity, no occupational hazards, does not smoke, denies alcohol abuse. Increased blood pressure (BP) has been noted for more than 10 years

up to 160/100 mm Hg. Art. no subjective sensations, does not regularly take antihypertensive therapy. Since 2013, I have been troubled by shortness of breath, weakness during physical activity, climbing stairs to the 2nd-3rd floor, periodic squeezing, stabbing pain in the heart area of ​​​​moderate intensity, occurring when walking 200 m or more, relieved by rest after 5-10 minutes . Over the course of two years, he notes a gradual increase in shortness of breath, up to orthopnea, the appearance of edema of the lower extremities, cardialgia with little physical activity.

On EchoCG from December 2013: left atrium (LA) - 5.0 cm; the final size of the left ventricle at relaxation (diastole) (LV EDR) is 7.3 cm; final size of the left ventricle during contraction (systole) (LV ESD) - 6.2 cm; left ventricular (LV) ejection fraction (EF) - 33%; interventricular septum (IVS) - 0.9 cm; posterior wall of the left ventricle (PLW) - 0.9 cm; the aorta is dilated, thickened; regurgitation on the mitral valve (MV) of the 2nd degree, on the tricuspid valve (TC) - 1st degree; dilatation of the atria and LV; diastolic dysfunction (DD) of both ventricles; LV systolic dysfunction; signs of pulmonary hypertension (PH).

In March 2014, he was hospitalized with a diagnosis of coronary artery disease. Ischemic dilatation cardiomyopathy. Decreased contractility of the LV myocardium (EF - 38%). Hypertension (HTN) stage 3, stage I, risk 4. On echocardiography from March 2014: LA - 4.9 cm, LV EDR -7.2 cm, LV ESD - 5.7 cm, LVEF - 38 %, IVS - 1.1 cm, LVSP - 1.0 cm, moderate PH, pulmonary artery systolic pressure (SPAP) - 46 mm Hg. Art.; regurgitation on the MV of the III degree, on the TC - II degree, on the pulmonary valve (PA) of the II degree, additional trabecula between the IVS and the lateral wall in the middle third of the LV; diastolic dysfunction (DD), collapse of the inferior vena cava (IVC) during inspiration more than 50%; dilatation of the cavities of the heart with a decrease in LV contractility.

Treatment included loop diuretics, aldosterone antagonists, nitrates, beta-blockers

During treatment, positive dynamics were noted, but after 4 months the patient stopped taking the drugs due to a significant improvement in his general condition. Over the course of a year, signs of heart failure progressed; attacks of suffocation and swelling in the legs reappeared.

Upon admission in 2016, the condition was moderate, orthopnea, pallor. Obesity of the 1st degree (BMI - 32.7 kg/m2), swelling up to the middle of the legs, respiratory rate (RR) - 24 per minute, breathing is harsh, weakened in the lower parts, single fine-bubbly moist rales. The left border of cardiac dullness is along the anterior axillary line. Heart sounds are muffled, the rhythm is regular, systolic murmur is at the apex and at Botkin's point. Heart rate - 120 beats/min, blood pressure - 140/90 mm Hg. Art., the dimensions of the liver according to Kurlov are 11x9x8 cm, the edge is smooth, painless, the spleen is not palpable.

Laboratory data: ESR - 25 mm/h; CBC: leukocytes - 4.7x109/l; erythrocytes - 4.11x1012/l; hemoglobin - 122 g/l; neutral - 72%; eosinophils - 3%; monocytes - 5%; lymphocytes - 20%; platelets - 339x109/l; OAM: straw yellow color, transparent, ud. weight - 1023, pH - 5.0; protein neg., sugar neg.; leukocytes - 1-2 per field of view; red blood cells - 0-1 in the field of view; epithelium - 1-2 in the field of view. Biochemical blood test: bilirubin - 9.0 µmol/l; glucose - 5.1 mmol/l; urea - 5.2 mmol/l; residual nitrogen - 26 mg/%; creatinine - 108.0 µmol/l; cholesterol - 3.7 mmol/l; ALT - 36.0 U/l; AST - 24.0 U/l; alkaline phosphatase - 199.0 IU/l; GTP gamma - 78.0 U/l; CPK - 83 U/l; LDH - 289.0 U/l. Coagulogram: fibrinogen A - 2.4 g/l; fibrinogen B neg.; APTT -32 s, INR - 1.79; PTV - 22 s; prothrombin - 100%.

The ECG (Fig. 1) shows sinus tachycardia 120 beats/min, a sharp deviation of the electrical axis of the heart (EOS) to the left (alpha angle minus 30°), ST depression of 1 mm

Rice. 1. ECG, March 2016

in V5, V6; RV5 > RV4. Incomplete blockade of the left bundle branch, mainly in the area of ​​the anterior branch; LV hypertrophy (LVH); decrease in QRS voltage in standard and enhanced limb leads.

On Holter ECG monitoring (HMECG): single ventricular extrasystoles; ST on isoline; T negative; rigid circadian rhythm profile. According to chest x-ray, diffuse enhancement of the pulmonary pattern due to the infiltrative-vascular component. Ultrasound examination of internal organs: signs of chronic cholecystitis, pancreatic steatosis, nodule of the right lobe of the thyroid gland, bilateral hydrothorax (free fluid about 50 ml).

On EchoCG: diffuse LV hypokinesis, areas of akinesia in the basal, middle and apical segments of the IVS, the apical segment of the lower wall; decrease in global contractile function of the ventricles: LVEF - 18% according to Simpson, RV Sm -8 cm/s; significant dilatation of all chambers (Fig. 2); LA - 5.3 cm; CDR - 7.5 cm; CSR - 6.9 cm (Fig. 3), left ventricular myocardial mass (LVMM) - 542 g; LVMI - 252 g/m2; right atrium (RA) volume -120 ml; anteroposterior size of the right ventricle (RVA) - 3.2 cm. Conclusion: systolic and diastolic dysfunction of both ventricles; severe left ventricular hypertrophy (LVH), enlargement of all chambers of the heart, significant insufficiency of the MV, TC and PA valve; pulmonary hypertension (PH) stage I, MPAP - 40 mm Hg. Art.

Based on the data obtained, the following diagnosis was made: “dilated cardiomyopathy. Incomplete blockade of the left bundle branch, predominantly of the anterior branch. Decreased global contractile function of both ventricles (LVEF 30% at discharge). Insufficiency of the mitral valve of the III degree, tricuspid valve of the II degree, pulmonary valve of the I-II degree. Hypertension stage 2, degree I, risk 4. Left ventricular hypertrophy. Chronic heart failure 2B, FC 4, episodes of cardiac asthma, pulmonary hypertension of the 1st degree, minor bilateral hydrothorax. Obesity of the 1st degree Focal changes in the right lobe of the thyroid gland. Chronic cholecystitis in the stage of incomplete remission, biliary sludge.”

Treatment included: bisoprolol 2.5 mg, perindo-pril 5 mg, furosemide 40 mg IV, torasemide 10 mg, ASA 100 mg, spironolactone 100 mg, ivabradine 7.5 mg. During treatment, positive dynamics were observed, tachycardia and shortness of breath decreased, and swelling disappeared. Test with a 6-minute walk of 450 m (CHF FC2). Positive dynamics on EchoCG: an increase in LVEF from 18 to 30%, a decrease in the volume of the right atrium, a decrease in MPAP from 40 to 36 mm Hg. Art. The patient was discharged on the 17th day in satisfactory condition. It is recommended to follow a diet limiting animal fats, table salt and easily digestible carbohydrates, control blood pressure and heart rate, continue treatment: bisoprolol 2.5 mg/day, perindopril 2.5 mg/day, ivabradine 7.5 mg/day, then

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Rasemide 5 mg/day, spironolactone 100 mg/day, ASA 100 mg/day. After discharge, the patient adhered to the above recommendations for a year. Over the past period, there has been no deterioration of the condition; he notes shortness of breath during moderate physical exertion (climbing stairs to the 2-3rd floor, accelerating his step), accompanied by discomfort in the precordial region.

At the time of inspection (April 2017), the condition was satisfactory. The skin has a physiological color. Obesity of the 1st degree, BMI - 30 kg/m2. There is no swelling. NPV - 17 per minute. Breathing is vesicular, no wheezing. The left border of the heart is along the anterior axillary line. The tones are muffled, the rhythm is correct, there is a soft systolic murmur at the apex and at Botkin's point. Heart rate - 80 beats/min, blood pressure - 120/85 mm Hg. Art. Liver according to Kurlov - 11*9*8 cm, the edge is smooth, painless. Survey data. ECG: sinus tachycardia - 80 beats/min, without significant dynamics compared to March 2016. Test with a 6-minute walk of 400 m (CHF FC2). XMECG: sinus rhythm, average heart rate - 74 beats/min; throughout the entire length the depression of the BG is up to 1 mm; negative T wave. EchoCG: dyskinesia of the apical segment of the IVS; hypokinesia of the lower, inferolateral, lateral medial segments and lower, lateral apical segments; severe concentric LVH; Type I DD of both ventricles; decreased contractility of both ventricles (LVEF - 30%, RV Sm - 12 cm/s). Dilatation of the left chambers. LA - 4.9 cm, EDR - 7.5 cm, ESR - 6.3 cm, LVMM - 522 g, LVMI - 243 g/m2, RA volume - 49 ml, RV PV - 2.6 cm; moderate insufficiency of MC, TC; signs of PH.

Results and its discussion. In this patient, it was not possible to accurately determine the cause of DCM. No hereditary burden was identified, but the influence of hypertension cannot be excluded. No data on the ischemic genesis of DCM have been identified: cardialgia can be explained by an increase in the size of the heart and its overload. The most likely is the idiopathic form of DCM. The clinical picture is characterized by biventricular CHF. At the beginning there was severe shortness of breath, general weakness, palpitations, and swelling of the legs. Incomplete blockade of the left bundle branch is also characteristic of DCM. At the same time, according to the results of CMECG, there are no arrhythmias, during the course of the disease the size of the heart has not increased significantly, the contractile function of the LV is sharply reduced, but is maintained by regular treatment. No further decrease in LVEF was observed; the prescribed drug treatment helped improve the patient’s quality of life. Taking into account the characteristics of the course of the disease, with strict adherence to treatment and regular outpatient monitoring of the condition, the prognosis is generally favorable.

Research transparency. The study had no sponsorship. The authors are solely responsible for submitting the final version of the manuscript for publication.

Declaration of financial and other relationships. All authors took part in the development of the concept, study design and

writing the manuscript. The final version of the manuscript was approved by all authors. The authors are not

received a fee for the research.

LITERATURE

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2. Kadish AH, Jacobson JT. Early Arrhythmic RiskAssessment in Idiopathic Cardiomyopathy: A Tincture of Time Can Be the Wrong Medicine. JACC Clin Electrophysiol. 2016; 2 (5): 544-545.

3. Piano MR. Alcoholic Cardiomyopathy: Is it Time for Genetic Testing? J Am Coll Cardiol. 2018; 71 (20): 2303-2305.

4. Wilcox JE, Hershberger RE. Genetic cardiomyopathies. Curr Opin Cardiol. 2018; 33 (3): 354-362.

5. Deo R. Alternative splicing, internal promoter, nonsense mediated decay, or all three; Explaining the distribution of truncation variants in titanium. Circ Cardiovascular Genet. 2016; 9: 419-425.

6. Elliott P, Andersson B, Arbustini E, et al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2008; 29: 270-276.

7. Arbustini E, Narula N, Dec G, et al. The MOGE(S) classification for a phenotype-genotype nomenclature of cardiomyopathy: endorsed by the World Heart Federation. J Am Coll Cardiol. 2013; 62(22):2046-2072.

8. Akinrinade O, Ollila L, Vattulainen S, et al. Genetics and genotype-phenotype correlations in Finnish patients with dilated cardiomyopathy. Eur Heart J. 2015; 36: 2327-2337.

9. Waseem M, Yaqoob N, Tariq MI, et al. Effects of Lower Limb Cycle Training on Echocardiographic

Parameters of Left Ventricle in Dilated Cardiomyopathy Patients. J Coll Physicians Surg Pak. 2018; 28 (5): 370373.

10. Yancy C, Jessup M, Bozkurt B, et al. 2013 ACCF/AHA guideline for the management of heart failure: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on practice guidelines. Circulation. 2013; 128: 18101852.

11. Ehlert F, Cannom D, Renfroe E, et al. Comparison of dilated cardiomyopathy and coronary artery disease in patients with life-threatening ventricular arrhythmias: differences in presentation and outcome in the AVID registry. Am Heart J 2001; 142:816-822.

12. Ingles J, Bagnall RD, Semsarian C. Genetic Testing for Cardiomyopathies in Clinical Practice. Heart Fail Clinic. 2018; 14 (2): 129-137.

13. Ware JS, Amor-Salamanca A, Tayal U, et al. Genetic Etiology for Alcohol-Induced Cardiac Toxicity. J Am Coll Cardiol. 2018; 71 (20): 2293-2302.

14. Arbustini E, Narula N, Tavazzi L, et al. The MOGE(S) Classification of Cardiomyopathy for Clinicians. J Am Coll Cardiol. 2014; 64: 304-318.

15. Masarone D, Kaski JP, Pacileo G, et al. Epidemiology and Clinical Aspects of Genetic Cardiomyopathies. Heart Fail Clinic. 2018; 14 (2): 119-128.

© PA. Mukhametshina, I.A. Gimaletdinova, N.B. Amirov, L.R Absyalyamova, O.Yu. Mikhoparova, A.R. Arslanova, 2018

UDC 616.127-005.8-079.4:616.37-002.1 DOI: 10.20969/VSKM.2018.11(4).119-124

DIFFICULTIES IN DIAGNOSIS OF ACUTE MYOCARDIAL INFARCTION IN A PATIENT WITH ACUTE PANCREATITIS

MUKHAMETSHINA GUZEL AGZAMOVNA, Ph.D. honey. Sciences, cardiologist of the cardiology department of the Clinical Hospital of the Medical Unit of the Ministry of Internal Affairs of the Russian Federation for the Republic of Tatarstan, Russia, 420059, Kazan, Orenburgsky tract, 132, tel. 8-929-723-12-91, e-mail: [email protected]

GIMALETDINOVA IRINA ANATOLYEVNA, gastroenterologist of the gastroenterological department of the Clinical Hospital of the Medical Unit of the Ministry of Internal Affairs of the Russian Federation for the Republic of Tatarstan, Russia, 420059, Kazan, Orenburgsky tract, 132, tel. 8-903-313-12-27, e-mail: [email protected]

AMIROV NAIL BAGAUVICH, ORCID ID: 0000-0003-0009-9103, SCOPUS Au^r ID: 7005357664; doc. honey. Sciences, Professor of the Department of General Medical Practice No. 1 of the Federal State Budgetary Educational Institution of Higher Education "Kazan State Medical University" of the Ministry of Health of Russia, Russia 420012, Kazan, st. Butlerova, 49, tel. 8-843-291-26-76, e-mail: [email protected]

ABSALYAMOVA LEILE RAVILOVNA, head. Gastroenterological Department of the Federal Public Health Institution “Clinical Hospital of the Medical Emergency Department of the Ministry of Internal Affairs of the Russian Federation for the Republic of Tatarstan”, Russia, 420059, Kazan, Orenburgsky tract, 132, tel. 8-965-608-72-73, e-mail: [email protected]

MIKHOPAROVA OLGA YURIEVNA, head. Department of Functional Diagnostics of the Federal Clinical Institution "Clinical Hospital of the Medical Emergency Department of the Ministry of Internal Affairs of the Russian Federation for the Republic of Tatarstan", Russia, 420059, Kazan, Orenburgsky tract, 132, tel. 8-937-525-5-261, e-mail: [email protected]

ARSLANOVA ALINA ROBERTOVNA, resident of the Department of Cardiology, X-ray Endovascular and Cardiovascular Surgery of the KSMA - branch of the Federal State Budgetary Educational Institution of Further Professional Education RMANPO of the Ministry of Health of Russia, Russia, 420012, Kazan, st. Mushtari, 11, tel. 8-937-773-02-45, e-mail: [email protected]

Abstract. The purpose of the study is to exchange experience in the differential diagnosis of acute myocardial infarction in a patient with acute pancreatitis. Material and methods. A clinical case is presented demonstrating the atypical development and course of acute myocardial infarction in a patient hospitalized at the Clinical Hospital of the Ministry of Internal Affairs of the Russian Federation for the Republic of Tatarstan with exacerbation of chronic pancreatitis, suffering from hypertension and diabetes mellitus. Results and its discussion. Depending on the clinical manifestations, the following forms of myocardial infarction are distinguished: asthmatic, cerebrovascular, arrhythmic and gastric. In particular, the clinical manifestations of the gastralgic variant are pain in the epigastric region, accompanied by various dyspeptic symptoms (heartburn, vomiting). In 5% of cases, the clinical manifestations of acute myocardial infarction and acute pancreatitis may be similar, which makes diagnosis difficult. Pain in acute pancreatitis is localized in the upper abdomen and can radiate to the left arm, shoulder and interscapular region, which may also be characteristic of acute myocardial infarction. However, the duration of the pain syndrome in acute myocardial infarction is several hours, and in acute pancreatitis the pain can last several days. In approximately 8% of cases with acute myocardial infarction, a single vomiting is observed, and in acute pancreatitis it can be repeated and accompanied by flatulence and stool disturbances. Absence of the Q wave on the electrocardiogram

CARDIOMYOPATHIES - section Education, INTERNAL DISEASES Relevance. Cardiomyopathies Remain One of the Least Studied.

Relevance. Cardiomyopathies remain one of the least studied cardiac diseases, being the object of an actively developing field of modern cardiology. Interest in the problem of studying myocardial diseases is explained by the need for further study of their etiology and pathogenesis, the diversity and nonspecificity of their clinical manifestations, and the presence of significant diagnostic and therapeutic problems. The constant increase in the incidence of various forms of cardiomyopathies is associated with the progress of modern diagnostic research methods. Over the last decade, a fundamentally new concept has been formed regarding the definition of the concept of “cardiomyopathies” and their place in the structure of heart diseases, which is associated with the achievements of medical genetics, morphology, immunology and molecular endocrinology. A reflection of the modern evolution of knowledge is the constant revision, updating and clarification of the corresponding concept and classification.

Terminology and classification. The term "cardiomyopathies" was first proposed by W. Brigden (1957) to refer to primary myocardial lesions of unknown etiology . causing dysfunction of the heart and not resulting from diseases of the coronary arteries, valvular apparatus, pericardium, systemic or pulmonary hypertension, as well as some rare variants of damage to the conduction system of the heart. This term has long been used in our country and abroad to refer to primary myocardial diseases of unknown etiology. According to the classification of J. Goodwin (1973), three forms of cardiomyopathies were identified: dilated (DCM), hypertrophic (HCM), and restrictive (RCMP).

Subsequently, thanks to the introduction of new diagnostic methods, it was possible to establish the genesis of some variants of cardiomyopathies. Thus, the causes of most cases of RCM have been established - endomyocardial fibrosis, Loeffler's disease, Fabry's disease, cardiac amyloidosis. In the development of DCM, the role of viral infection, autoimmune processes, heredity, etc. has been proven. And thus, the designation of cardiomyopathies as diseases of unknown etiology has largely lost its original meaning. It has been demonstrated that with known diseases of the internal organs of an infectious, metabolic, toxic and other nature, damage to the myocardium occurs with disruption of its functions, reminiscent of the features of cardiomyopathy.

According to the classification of cardiomyopathies (WHO, 1995), cardiomyopathies are defined as diseases of the myocardium associated with its dysfunction. They are divided into dilated (DCM), hypertrophic (HCM), restrictive (RCMP), arrhythmogenic right ventricular and unclassified cardiomyopathies. Moreover, each of the cardiomyopathies does not characterize a separate nosological form, but represents a clearly defined syndrome, including a certain morphofunctional and clinical-instrumental symptom complex, characteristic of a heterogeneous group of myocardial diseases.

Rice. 2. Types of cardiomyopathies. A – normal, B – DCM,

B – restrictive cardiomyopathy,

G – hypertrophic cardiomyopathy

Table 28

Classifications of cardiomyopathies (WHO, 1995)

Cardiomyopathy

Cardiomyopathy is understood as primary or secondary damage to the heart muscle, the cause of which is not an inflammatory process, tumor or damage to the blood vessels of the heart. This is a collective name for a whole group of myocardial diseases with an unknown etiology or an established cause.

Classification of cardiomyopathies

1. Primary (idiopathic)
   • Dilatational. The thickness of the walls of the ventricles is not increased, but expansion of the cavities of the heart occurs, which leads to systolic dysfunction, impaired cardiac output and the development of heart failure. Sometimes this type is also considered ischemic cardiomyopathy, which occurs in people suffering from coronary heart disease.
   • Hypertrophic. Thickening of the wall of one of the ventricles or both at once, more than 1.5 cm. This is an intrauterine hereditary or acquired defect; can be symmetrical or asymmetrical (more common), as well as obstructive and non-obstructive.
   • Restrictive. It is rare, and in turn is divided into obliterating and diffuse. With this cardiomyopathy, a violation of the contractile function of the myocardium occurs, due to which there is an insufficient volume of blood in the chambers of the heart, and the load on the atria greatly increases.
   • Arrhythmogenic right ventricular dysplasia. A rare hereditary disease also called Fontan disease. Necrosis of myocardial tissue due to large amounts of fatty deposits leads to serious forms of arrhythmia or cardiac arrest.
2. Secondary (cause known)
   • Alcoholic
   • Diabetic
   • Thyrotoxic
   • Stressful

Causes

If we are talking about a secondary disease, then the etiology is known - as can be seen from the classification, it can be alcohol, a severe stressful situation, diabetes, etc. With the primary type, the cause often remains unidentified; among the possible ones, scientists name the following:

• Genetic predisposition, inherited defect, gene mutations,
• Exogenous: viruses (Coxsackie, herpes, influenza, enteroviruses, etc.), bacteria, fungi, exposure to toxic substances (alcohol, medications, heavy metals), etc.
• Autoimmune diseases
• Metabolic and nutritional disorders, endocrine diseases
• Muscular dystrophies
• Pheochromocytoma

Complications and prognosis

Cardiomyopathy leads to the development of progressive heart failure, as well as such serious consequences as arrhythmia. thromboembolism. All this threatens sudden death. A favorable prognosis is observed in patients who have undergone competent systematic treatment, surgery, and rehabilitation therapy.

Our center has all the necessary equipment for a thorough diagnosis of cardiomyopathies. For effective surgical treatment, we refer patients to our foreign partners. The rehabilitation course and the necessary follow-up can also be completed at our center.

– this is a group of pathologies that are accompanied by disruptions in the functioning of the heart muscle. There are several mechanisms of myocardial damage that unite such diseases.

The causes of cardiomyopathy are most often unclear, and to make a diagnosis it is necessary to ensure the absence of congenital defects. There are different types of such pathology, and the classification of cardiomyopathies depends on the characteristics of circulatory disorders in the heart.

Cardiomyopathy is a collective term for a group of myocardial pathologies of unknown origin. The basis of such diseases is dystrophic and sclerotic processes in heart cells - cardiomyocytes. With a disorder such as cardiomyopathy, in most cases the functioning of the ventricles of the heart changes.

To date, it has not been possible to establish the true causes of cardiomyopathies. Experts say that such pathologies can progress due to mutational changes in genes or as a result of the active development of existing diseases in a person.

T These diseases can be diagnosed in people of any age, but in children they are detected much less frequently than in adults.

This is due to the fact that this group of diseases is hereditary in nature, and problems with heart function can already appear in young children.

Causes and symptoms

Cardiomyopathies include any pathology that is accompanied by damage to the heart muscle. In fact, there are many reasons that can provoke the development of such a disease.

In the event that malfunctions in the functioning of the myocardium are a consequence of other diseases identified in a person, then they speak of secondary or specific cardiomyopathies. Otherwise, the root cause of the development of cardiomyopathy cannot be identified, and doctors talk about the primary form of the disease.

The most common causes of cardiomyopathy are:

1. congenital disorders often become one of the causes of the development of cardiomyopathies, that is, even at the stage of embryonic tissue formation, various malfunctions occur
2. acquired causes are a consequence of the progression in the human body of various viruses, toxic substances and problems with metabolic processes
3. mixed causes combine several factors that cause the development of cardiomyopathy

The disease is often detected in children, and it can be either congenital or acquired. Secondary pathology develops as a consequence of exposure to external and internal factors on the organ, as well as another disease.

The most common secondary causes are:

• long-term diets, which reduce the content of nutrients and vitamins in the human body
• improper and irrational nutrition, excess weight and pathologies of the gastrointestinal tract
• disruptions in metabolism in the myocardium as a result of pathologies of the endocrine system
• drinking large amounts of alcoholic beverages
• taking medications and especially antitumor drugs
• accumulation of various pathological inclusions in cells

In recent years, mortality due to secondary cardiomyopathy has become common. The reasons for the development of such pathology are varied and in each individual case a high-quality and professional examination is required.

The signs of cardiomyopathy have many similarities with the clinical picture of progressive cardiomyopathy.

Medical practice shows that with this disease, patients most often complain of:

• increased body fatigue
• constant swelling of the limbs
• paroxysmal pain in the heart area
• weakness and dizziness
• attacks of shortness of breath
• sleep problems

The appearance of such symptoms indicates that a person has disturbances in the contraction of the heart and problems with its blood supply. If such signs occur, you should seek help from a doctor as soon as possible, which will allow you to take timely measures.

Classification

When studying such cardiac pathologies, scientists were able to establish their individual classification, according to which they are divided into two types: primary and secondary.

If it is not possible to identify the cause of the development of the pathology, then the primary ailment is diagnosed, which is classified into the following types:

• Restrictive cardiomyopathy. This disorder is accompanied by myocardial rigidity, when the chambers of the heart are filled with blood in a small volume. The consequence of this pathological condition is the occurrence of diastolic dysfunction and. The main cause of restrictive cardiomyopathy is considered to be genetic mutations.
• . This pathology is characterized by thickening of the wall of one of the ventricles without a pronounced expansion of the cardiac cavities. The main reason for the development of this disease is various genetic defects. Hypertrophic cardiomyopathy is divided into symmetrical, obstructive, non-obstructive, apical and asymmetrical. Medical practice shows that most often patients are diagnosed with hypertrophic obstructive cardiomyopathy.
• . This type of disease is characterized by various disruptions in the contractile function of the heart due to dilatation of its chambers. The development of this type of pathology is explained by immune and genetic disorders.

If the diagnosis failed to identify the cause of the disease, then they speak of secondary cardiomyopathy. In fact, it is more dangerous to human life than the primary one. Experts say that often the main cause of death is secondary cardiomyopathy, which progresses in a severe form in the human body.

More information about cardiomyopathy can be found in the video:

The secondary type of disease is divided into the following types:

1. Alcoholic cardiomyopathy. This type of pathology is characterized by severe organ damage as a result of prolonged use of alcoholic beverages. A characteristic manifestation of this disease is heart failure, and sometimes myocardial ischemia. Alcoholic cardiomyopathy is often the cause of death.
2. Toxic cardiomyopathy. The appearance of problems in the functioning of the heart is associated with prolonged exposure to any strong toxins on the human body.
3. Ischemic cardiomyopathy. Myocardial disease provoked by various diffuse morphofunctional disorders that arise as a result of chronic or acute myocardial ischemia. This type of disease is characterized by heart failure and dilatation of the heart chambers.
4. Dyshormonal cardiomyopathy. This pathology is accompanied by non-inflammatory damage to the heart muscle, which occurs when there is insufficient content of sex hormones due to various metabolic disorders in the myocardium.
5. Dysmetabolic cardiomyopathy. Such heart failure begins to progress against the background of prolonged overstrain of the organ and as a result of a violation of the body’s homeostasis. Most often, this type of pathology is diagnosed in young patients who are actively involved in sports.
6. Metabolic cardiomyopathy. This pathology is characterized by myocardial dystrophy and insufficiency of cardiac function, which occurs as a result of failures in metabolic and energy-generating processes in the myocardium.
7. Takotsubo cardiomyopathy. This disease is a non-ischemic type, expressed by heart failure and pain in the chest. The main cause of development is considered to be severe emotional stress, and a sudden decrease in myocardial contractility is observed.

In addition to all the listed types, there is another form of this pathology - unspecified cardiomyopathy. It is characterized by the occurrence of some specific conditions that cannot be attributed to any of the existing types.

Diagnosis of pathology and treatment

When diagnosing cardiomyopathies, special attention is paid to the clinical picture of the disease and the indicators of the instrumental studies performed. Today, it is considered the main way to detect various cardiac disorders.

In order to diagnose a specific type, doctors widely use the following diagnostic methods:

• radiography
• electrocardiogram
• CT scan
• probing

When a patient is diagnosed with one or another type of disease, drug therapy for cardiomyopathy is first selected. Diuretics are prescribed to reduce pulmonary and systemic venous congestion.

Cardiac glycosides are used to identify problems with myocardial contractility and disruption of its pumping function.

Taking antiarrhythmic drugs helps correct the heart rhythm, and treatment with anticoagulants and antiplatelet agents helps eliminate thromboembolic complications.

Taking into account the severity of the pathology and its nature, other more radical methods of therapy can be used:

1. surgical intervention can be performed for various types of cardiomyopathy, but in most cases it is used to eliminate hypertrophic obstructive heart pathology
2. treatment using stem cells is most often carried out when dilated cardiomyopathy is detected in a patient
3. Heart transplantation is performed only in extremely advanced cases

To date, medicine has not yet been able to develop a universal treatment regimen for such complex heart diseases. Timely medical care makes it easier for patients to live and prolongs it for a long time, but at the same time, the mortality rate remains at a high level.