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is an arrhythmia (most often AF) or thromboembolic complications

yami.

As a rule, patients with dilated cardiomyopathy seek medical help in

due to HF decompensation, therefore, on examination, shortness of breath, acrocyanosis, peripheral edema, jugular vein pulsation are noted. The boundaries of relative cardiac

dullness are expanded, the first tone at the apex of the heart is weakened, pathological tones are often hea systolic murmurs of mitral and tricuspid regurgitation. In patients with thromboembolism in

the pulmonary circulation system, there is an accent of II tone on the pulmonary artery. and diastolic murmur due to pulmonary valve insufficiency. Often

revealed signs of fluid accumulation in the pleural cavity, liver enlargement, ascites.

Laboratory and instrumental diagnostics. When analyzing the blood of patients with dilated cardiomyopathy, nonspecific changes can be detected due to complications of the disease: pulmonary embolism with the development of pneumonia infarction, renal and hepatic insufficiency due to severe disorders

central hemodynamics.

Electrocardiographic examination in many patients reveals rhythm disturbances (AF, atrial and ventricular premature beats, non-sustained VT) and conduction (more often total intraventricular block). In most cases, there is a decrease

R-wave amplitudes in standard and anterior chest leads, biphasic and inversion

T wave, sometimes - the appearance of a Q wave in leads V1-V3.

Chest x-ray reveals marked cardiomegaly, signs of congestion in the lungs and the presence of fluid in the pleural cavity.

Echocardiography in everyday clinical practice is the main method for verifying the diagnosis of dilated cardiomyopathy, but the changes that are detected by ultrasound examination of the heart are also not specific: dilatation of the cavities, mitral and tricuspid regurgitation, signs of systolic, diastolic dysfunction and

pulmonary hypertension, the presence of intracavitary thrombi.

Radionuclide ventriculography and magnetic resonance imaging of the heart can be used to diagnose dilated cardiomyopathy. In modern specialized

medical centers to clarify the etiology of cardiomegaly and identify variants of heart muscle damage, in which it is possible to use specific pharmacotherapy (amyloidosis, hemochromatosis, sarcoidosis, viral and immune heart damage).

genesis), myocardial biopsy is used.

Diagnosis. The diagnosis indicates the underlying disease and its complications.

Diagnosis example:

dilated cardiomyopathy. Atrial fibrillation (permanent

the form). Thromboembolism of small branches of the pulmonary artery 22.03.2010.

Chronic heart failure IIB Art., III f. to.

differential diagnosis. The diagnosis of dilated cardiomyopathy is established by

exclusion of known causes of cardiomegaly. Dilatation of the heart and the development of chronic heart failure

are most often caused by coronary artery disease, hypertension, non-rheumatic myocarditis, malformations heart, tachyarrhythmias, diabetes mellitus, chronic intoxications (in particular,

alcohol abuse), infections.

The painless form of coronary artery disease in most cases is accompanied by

atherogenic changes in the blood lipid spectrum and transient changes in the terminal part of the ventricul ischemic complex, which can be detected by Holter ECG monitoring. Electrocardiographic

signs of a postinfarction scar in the form of a pathological Q wave or

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QS forms, and with echocardiography - local contractility disorders

myocardium. In doubtful cases, if the patient'scondition allows, stress tests are carried out. In patients with severe HF, positron emission tomography can be used to identify CAD-specific perfusion and myocardial metabolism disorders, and multilayer helical computed tomography can be used to assess the state of the coronary arteries. tomography.

Inflammatory genesis of cardiomegaly can be suspected in cases where the onset of the disease is associated with any infection, vaccination, medication, systemic allergy. Arguments in favor of non-rheumatic myocarditis can be changes in the blood test characteristic of inflammation, an increase in the concentration of the creatine phosphokinase MB fraction, cardiac fractions of lactate dehydrogenase, aspartic aminotransferase and cardiac troponins. An increase in the titer of antiviral antibodies - an important diagnostic criterion for current viral myocarditis. To confirm the diagnosis of myocarditis, endomyocardial biopsy, cardiac tomoscintigraphy, magnetic resonance imaging of the heart with contrast are used. At the same time, it is impossible not to take into account that in many cases it is the viral infection that becomes the trigger implementation of genetic disorders that cause the formation of dilated cardiomyopathy.

Diagnosis of alcoholic heart disease is based on the results of questioning patient and analysis of changes in clinical and instrumental indicators characterizing cardiac activity, after the patient refuses to drink alcohol.

Flow. Dilated cardiomyopathy is usually diagnosed after the onset of symptoms. CH. Periods of decompensation of the function of the heart as a pump alternate with periods of a relatively stable state, but HF in patients with dilated cardiomyopathy progresses steadily. Thromboembolic complications and arrhythmias usually become additional reasons for the deterioration of the condition of patients.

Forecast and outcomes. Dilated cardiomyopathy has a poor prognosis. The period of time from the appearance of the first signs of HF to the death of the patient is not more than 3 - 5 years. The most common causes of death are progressive heart failure, thromboembolism, and life-threatening arrhythmias.

Treatment. Drug treatment of patients with dilated cardiomyopathy is aimed at prevention of HF progression and correction of cardiac arrhythmias. Apply

ACE inhibitors and ÿ- blockers, in the presence of stagnation - diuretics and spironolactone. In cases of insufficient effectiveness of the listed drugs and severe systolic dysfunction, cardiac glycosides may be used. drugs

of choice for the correction of AF and ventricular arrhythmias are cordarone and

sotalol. Indications for the use of anticoagulants as part of the pharmacotherapy of dilated CMP with to reduce the risk of thromboembolic complications are AF, thromboembolism in

history and the presence of blood clots in the cavities of the heart.

In the treatment of patients with persistent viral infection, interferon is used, which promotes the elimination of cardiotropic viruses from the myocardium. Interferon

treatment should be combined with immunosorption. This method removes from the blood autoantibodies and circulating immune complexes, which contributes to the suppression

of autoimmune and inflammatory mechanisms of the progression of myocardial systolic dysfunction card.

A very promising method for the treatment of patients with severe HF and impaired Intraventricular conduction is a three-chamber pacemaker, which allows you to resynchronize the work of the heart and thereby improve its pumping function and

increase the life expectancy of the patient. In patients with low (less than 35%) ejection fraction of the ventricle and a high risk of life-threatening ventricular arrhythmias are more effective

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than the use of drugs, a way to prevent sudden death

implantation of a cardioverter-defibrillator. In recent years, in the treatment of patients with dilated cardiomyopathy with appropriate indications, both types are increasingly used. the above implantable devices.

The surgical treatment for dilated cardiomyopathy is heart transplantation.

1.9.3. Restrictive cardiomyopathy

Definition. Restrictive cardiomyopathy is a heart disease characterized by a predominant violation of the filling of one or both ventricles with blood due to

increased stiffness of the myocardium and / or endocardium in the absence of an increase in the volume of the stomach

daughters.

Prevalence. Restrictive CMP is less common than other types of CMP.

Some forms of restrictive cardiomyopathy are endomyocardial fibrosis and fibroplastic Loeffler'seosinophilic endomyocarditis is extremely rare in Russia, but in

at the same time are endemic to some tropical and subtropical countries.

Etiology. Restrictive cardiomyopathy develops as a result of damage to the endocardium (fibrosis, fibroelastosis, often in combination with the formation of intracavitary thrombi), endomyocardium (fibrosis, infiltration), myocardium (fibrosis, infiltration, hypertrophy), subepicardium (infiltration).

The cause of familial (hereditary) restrictive CMP is genetic disorders - mutations in the cardiac troponin I gene. Infiltration of the heart wall in the majority

cases is represented by amyloid masses, iron deposits, glycogen, sarcoid

granulomas, clusters of eosinophils (hypereosinophilic conditions), tumor

cells. Thus, the main diseases that cause the formation of restrictive cardiomyopathy are amyloidosis, hemochromatosis, sarcoidosis, systemic scleroderma, idiopathic and specific (i.e., associated with parasitic invasions, the use

drugs, bronchial asthma, leukemia, etc.) hypereosinophilia, tumor processes. Restrictive ventricular dysfunctions are observed in hypertrophic and dilated cardiomyopathy, as well as in myocardial damage associated with hypertension, diabetes mellitus, and other diseases.

Pathogenesis. Fibrosis and infiltration of the myocardium with pathological substances or cellular elements cause a decrease in its elasticity. Violation of the relaxation of the stomach

daughters of the heart leads to an increase in intraventricular pressure, resulting in an increase in

pressure in the atria, and then in the veins of the systemic and pulmonary circulation,

symptoms of congestive heart failure appear. An increase in the wall thickness of the left ventricle is accomp a decrease in the size of its cavity, which leads to a decrease in stroke volume and becomes

additional cause of HF progression.

Morphology. Morphological changes in the heart in restrictive cardiomyopathy are diverse. Endomyocardial fibrosis is characterized by marked thickening of the endocardium with proliferation of connective tissue in the endocardium and subendocardial layer of the myocardium, calcification of the mitral and aortic valves. Often found in the ventricular cavities

blood clots. With Loeffler'sendomyocarditis, eosinophilic cells are found in the endocardium and myocardium. infiltrates, small foci of necrosis, areas of inflammatory infiltration and fibrosis. These

changes are often accompanied by the formation of intracavitary thrombi. Are amazed mainly the ventricles of the heart. Both bilateral and unilateral lesions are possible.

The main cause of heart damage in hypereosinophilic syndromes is considered to be the effect of cytotoxic proteins, products of eosinophil degranulation.

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Amyloid masses are deposited in the myocardium of the ventricles and atria. Sometimes it'stime

the pericardium, endocardium, valves and papillary muscles, as well as the veins and arteries of the heart, are squeezed.

In sarcoidosis, epithelioid cell granulomas and foci of fibrosis are formed in the myocardium, which are characteristic of this disease. There may be a small amount of effusion in

pericardial cavities. Hemochromatosis is characterized by the accumulation of iron in the tissues of various

organs, including the heart. The process is localized mainly in the myocardium of the left ventricle. First, its subepicardial, and then the subendocardial layer is affected.

The cytotoxic effect of excess iron is manifested by the degeneration of cardiomyocytes, followed by the growth of fibrous tissue. Carcinoid damage to the heart is caused by the action of biologically active substances secreted by the cells of certain tumors.

intestines. The right ventricle is predominantly affected - endocardial fibrosis develops with the formation of defects (stenosis or insufficiency) of the tricuspid valve and valve

pulmonary artery. Metastatic myocardial damage is characterized by accumulations of tumor cells with perifocal inflammation, damage to the tricuspid valve and valve

pulmonary artery by the formation of heart defects, the development of exudative pericarditis with

metastatic lesions of the pericardium.

clinical picture. Leading clinical manifestation of restrictive cardiomyopathy

is a symptom complex of chronic heart failure. Impaired function of the heart as a pump is due to diastolic dysfunction of the left and/or right ventricle. In some patients, the conduction system of the heart is involved in the process of fibrosis, which leads to the formation of AV blockade.

On examination and physical examination, more or less pronounced signs of heart failure are revealed: shortness of breath, cyanosis of the lips, peripheral edema, tachycardia, hepatomegaly, ascites. A slight expansion of the boundaries of relative cardiac dullness is determined

both left and right chambers of the heart, a decrease in the volume of the I tone at the top. In some patients, pathological III and IV tones are auscultated. With damage to the mitral and

tricuspid valves appear systolic murmurs of regurgitation. Congestive rales are heard in the lungs.

Additional symptoms are determined by etiopathogenetic features specific variant of the disease. For Loeffler'sfibroplastic endomyocarditis

damage is characteristic not only of the heart, but also of other organs - the lungs, bone marrow, central nervous system, kidneys, liver, gastrointestinal tract, weight loss,

fever, skin rash.

Laboratory and instrumental diagnostics. When examining blood in patients with idiopathic hypereosinophilic syndromes (in particular, with Loeffler'sfibroplastic endomyocarditis), persistent eosinophilia is detected. For primary

amyloidosis is characterized by dysproteinemia in the form of an increase in the concentration of monoclonal

immunoglobulins or their light chains. Hemochromatosis is manifested by an increase in the content iron in the blood plasma and the degree of saturation of transferrin, as well as a decrease in the ironbinding capacity of serum.

ECG changes are nonspecific and in most patients are expressed in a decrease in the voltage of the teeth of the ventricular complex, rhythm and conduction disturbances. Some patients show electrocardiographic signs of ventricular hypertrophy and increased

atria.

X-ray examination allows, in some cases, to reveal a slight cardiomegaly and a decrease in pulsation along the contour of the heart, as well as due to heart failure.

congestive changes in the lungs. Patients with sarcoidosis have symmetrical bilateral enlargement of intrathoracic lymph nodes.

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Echocardiographic examination reveals: thickening of the walls of the ventricles

in the absence of expansion, and sometimes a decrease in the size of their cavities, normal ejection fraction of the left ventricle, atrial dilatation, often - deformation of the valves with signs of mitral and tricuspid regurgitation. Using the ultrasonic method

it is possible to identify intracavitary thrombi, and sometimes thickening of the endocardium.

Doppler echocardiography reveals a pronounced violation of the diastolic function of the ventricles. Less comm assessment of diastolic function, cardiac catheterization with radiopaque

ventriculography or radioisotope ventriculography.

To clarify the genesis of restrictive cardiomyopathy and conduct differential diagnosis (for example, with constrictive pericarditis), a transvenous catheter

endomyocardial biopsy. Biopsy analysis reveals amyloid deposits, signs endomyocardial fibrosis, hemochromatosis, metastatic lesions, etc.

Diagnosis. Depending on the clinical situation, the term "restrictive cardiomyopathy" may be indicated or not indicated in the diagnosis. In cases of disease, the main manifestation which is the restrictive physiology of the left ventricle, this term is usually

goes down. In diseases for which the development of restrictive cardiomyopathy is not mandatory, this term should be included in the diagnostic formula.

Diagnosis examples:

1. Loeffler'sfibroplastic endomyocarditis. Chronic heart failure IIB Art., III f. to.

2. Primary amyloidosis with damage to the heart. Restrictive cardiomyopathy. Atrioventricular block I stage. Chronic cardiac insufficiency IIA Art., II f. to.

differential diagnosis. Restrictive cardiomyopathy and constrictive pericarditis present with similar clinical symptoms. Differential diagnosis of these

diseases is carried out using imaging research methods - echocardiography, computed tomography and magnetic resonance imaging, which allow assessing the thickness of the pericardium. Thickening of the pericardium, often with calcium deposits

is a characteristic feature of constrictive pericarditis.

Certain difficulties are presented by the diagnosis of diseases in which the restrictive

active cardiomyopathy develops as a consequence of the underlying pathological process.

Amyloidosis is characterized by the presence of signs of a systemic disease: hepatoand splenomegaly, enlargement of the tongue, proteinuria, often up to the development of

nephrotic syndrome, dysproteinemia, neuropathy, hypothyroidism, adrenal insufficiency. Diagnosis Amyloidosis can be confirmed by biopsy of the gingival mucosa, fat cells, kidneys, and liver.

In cases of isolated cardiac amyloidosis, the diagnosis is made

according to the results of myocardial biopsy.

The systemic nature of the disease is typical for hemochromatosis. In patients, in most cases, a combination of melasma, cirrhosis of the liver and diabetes mellitus is detected. Dia

the diagnosis is confirmed by the results of the analysis of the content of iron in the blood plasma, the degree of nasa

transferrin and iron-binding capacity of serum. In doubtful cases

a biopsy of the liver, skin, myocardium is performed.

Sarcoidosis is manifested by nonspecific symptoms: weakness, subfebrile fever, shortness of breath, weight loss. Some patients develop

nodular erythema. Diagnosis is mainly based on chest x-ray, which shows symmetrical bilateral intrathoracic lymph node enlargement. To verify the diagnosis, a myocardial biopsy is performed.

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Flow. Restrictive cardiomyopathy is characterized by a progressive increase in diastolic ventricular dysfunction and clinical symptoms of heart failure.

Forecast and outcomes. The prognosis is unfavorable. The cause of death in patients with restrictive cardiomyopathy is most often progressive HF, ventricular arrhythmias

heart and thromboembolism.

Treatment. Symptomatic therapy is aimed at reducing the severity and inhibiting the progression of HF. ACE inhibitors, ÿ- blockers and diuretics are mainly used. The use of cardiac glycosides is impractical due to the lack of

systolic dysfunction. According to the indications, antiarrhythmic drugs are used. For prevention of heart damage in hypereosinophilic syndromes, a rapid

suppression of eosinophilia. For this purpose, corticosteroids and cytostatics are used. Sick with intracavitary thrombi, anticoagulants are prescribed to prevent embolism. At

primary amyloidosis, certain hopes for improvement in cardiac function are associated with cytostatic therapy and stem cell transplantation. main direction

Treatment of sarcoidosis remains the use of glucocorticoids. Patients with hemochromatosis produce regular bloodletting. The above methods of treatment in most cases

give only a short-term improvement in the condition of patients.

1.9.4. Arrhythmogenic right ventricular dysplasia

Definition. Arrhythmogenic right ventricular dysplasia (or arrhythmogenic cardiomyopathy) - a disease characterized by progressive fibro-fatty degeneration of the myocardium of the free

wall of the right ventricle with relative intact interventricular

partitions.

Prevalence. There are no exact data on the prevalence of arrhythmogenic cardiomyopathy.

It is believed that the frequency of this pathology is 1: 5000. In young people, arrhythmogenic cardiomyopathy

diagnosed more often than in middle-aged and elderly people. This disease is considered as one of the leading causes of sudden death under the age of 35 years. In men, aritis mogenic CMP is detected 2.5-3 times more often than in women.

Etiology. Arrhythmogenic cardiomyopathy is associated with mutations in the genes that code for the synthesis

proteins that provide the skeletal function of cardiomyocytes. In most cases, arrythmogenic CMP is inherited in an autosomal dominant manner. The familial nature of the disease is confirmed in almost half of the cases.

Pathogenesis. Changes in the myocardium of the right ventricle are localized mainly in within the so-called "triangle of dysplasia": inlet tract - apex - outlet

tract. The interventricular septum and left ventricle are affected rarely and to a lesser extent. Due to fatty and fibrous degeneration of the myocardium, its thinning occurs,

aneurysms are formed. With the formation of structural changes in the myocardium, prerequisites are created for the occurrence of right ventricular arrhythmias and right ventricular heart failure.

Morphology. Histological examination reveals signs of atrophy and apoptosis

cardiomyocytes, their replacement by cells of adipose and connective tissue. Often in the myocardium foci of inflammation in the form of lymphocytic infiltration and other signs of infection with cardiotropic viruses are detected. The wall of the right ventricle is thin and often has

numerous aneurysmal bulges. Degeneration of the myocardial wall of the right ventricle begins with the subepicardial layer and gradually spreads to the side

subendocardium.

clinical picture. Among the main clinical manifestations of arrhythmogenic

CMP includes syncope and palpitations due to paroxysmal right ventricular tachycardia. As the morphological changes in the myocardium increase,

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HF symptoms progress with predominant stagnation in the systemic circulation.

On examination, there is swelling of the cervical veins, cyanosis of the lips, peripheral edema, hepatomegaly. Examination of the heart reveals an expansion of the boundaries of relative cardiac dullness to the right, retrosternal pulsation and murmur of tricuspid regurgitation are

often noted. At a late stage of the disease, cardiomegaly is formed and the clinical picture of severe biventricular insufficiency with stagnation in both circles of blood circulation. dilatation

right ventricle and arrhythmias contribute to the formation of intraventricular thrombi. At some patients experience thromboembolism - mainly in the pulmonary artery system.

Laboratory and instrumental diagnostics. Blood tests do not reveal any changes specific to arrhythmogenic cardiomyopathy.

Electrocardiographic examination usually reveals signs of incomplete

blockade of the right leg of the bundle of His. Outside of attacks of tachycardia, many patients have an expansion of the QRS complex up to 110 ms or more in leads V1–V3, specific for arrhythmogenic cardiomyopathy. Somewhat less frequently, the so-called epsilon wave is

detected in the form of low-amplitude oscillations following the QRS complex. in the right chest negative T waves are often detected and sometimes a small (up to 0.1 mV) rise in the segment ST. There is frequent ventricular extrasystole, unstable VT, AF.

Echocardiography makes it possible to assess the size, thickness, and kinetics of the walls of the right

ventricle, which is of great importance for the diagnosis of arrhythmogenic cardiomyopathy. For the purpose of evaluating time

measurements of the cavity of the right ventricle and the movement of its walls can be used X-ray

trust ventriculography or radionuclide ventriculography. Magnetic resonance

tomography allows you to assess the density of the ventricular wall, in particular, to distinguish between muscular

tissue from adipose. Although fatty infiltration of the myocardium is not a specific sign of

arrhythmogenic cardiomyopathy, the detection of accumulations of adipose tissue in the wall of the heart can s

an important argument in favor of this diagnosis.

Endomyocardial biopsy is rarely used to diagnose arrhythmogenic cardiomyopathy, since myocardial damage is focal in nature and is localized mainly in the sub-epicardial layer.

An important method for obtaining diagnostic information and assessing the condition of patients arrhythmogenic cardiomyopathy is an electrophysiological (EP) study. Application of this

method is considered mandatory when examining patients with non-sustained and sustained VT, ventricular fibrillation, syncope, systolic dysfunction of the right ventricle, as well as persons who have experienced clinical death due to circulatory arrest, and patients whose close relatives have been diagnosed with arrhythmogenic cardiomyopathy

with life-threatening cardiac arrhythmias. The EF study makes it possible to assess the inducibility of tachycardia, the mechanism, hemodynamic significance, and sensitivity of arrhythmias to drug and non-drug antiarrhythmic effects.

The diagnosis of arrhythmogenic cardiomyopathy is formed from the names of the underlying disease and its complications.

Diagnosis example:

Arrhythmogenic dysplasia of the right ventricle. unstable ventricular tachycardia. Chronic heart failure IIA stage, II

f. to.

differential diagnosis. The main features, the presence of which allows

to suspect the relationship of life-threatening ventricular arrhythmias with arrhythmogenic cardiomyopathy, are:

family history or early onset of unusual heart disease, syncope, arrhythmia

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episodes, tachycardia, structural changes in the right ventricle, right ventricular

Heart failure, changes in ventricular complexes in the right chest ECG leads. EF-study allows to differentiate tachycardias associated with arrhythmogenic

cardiomyopathy from tachycardias caused by the presence of additional pathways or dissociation of the AV connection, as well as tachycardia of the outflow tract of the right ventricle.

Certain difficulties are presented by the differential diagnosis of arrhythmogenic CMP and Brugada syndrome, which is characterized by similar clinical symptoms (fainting, increased risk of sudden death due to paroxysms of polymorphic VT

and ventricular fibrillation) and similar ECG changes (slight segment elevation

ST in leads V1– V3 with a negative T wave, right bundle branch block). Brugada syndrome is associated with a mutation in the gene encoding the function of sodium channels. Expressed structural changes in the heart, as a rule, are not observed. The absence of morphological changes in the right ventricle is considered as the main feature that makes it possible to distinguish Brugada syndrome, which is called "functional arrhythmogenic cardiomyopathy", from arrhythmogenic right ventricular dysplasia.

Flow. Over time, in patients with arrhythmogenic cardiomyopathy, there is an increase structural and functional disorders of the right ventricular myocardium, increased risk of sudden arrhythmic death and progression of right ventricular, and later biventricular HF.

Forecast and outcomes. The prognosis is unfavorable. Highest risk of sudden death considered for patients who have recorded episodes of VT in combination with at least one of the following factors: death of close relatives at a young age from arrhythmogenic

CMP, symptoms of right ventricular failure, ST segment elevation in the right chest leads more than 0.1 mV.

Treatment of patients with arrhythmogenic cardiomyopathy is aimed at solving two problems:

and prevention of VT paroxysms; relief of symptoms and slowing of progression CH.

In case of unstable VT, which does not cause pronounced disorders of the central hemodynamics, the tactics of drug treatment is used, which consists in empirical selection of an effective antiarrhythmic drug. Used for drug therapy

ÿ- blockers, sotalol, amiodarone, novocainamide, propafenone. The choice of drug is carried out using EF testing. In cases of drug failure or intolerance, as well as in patients with lifethreatening arrhythmias, ablation of arrhythmogenic substrates or implantation of an cardioverter-defibrillator is used.

For the treatment of heart failure, ACE inhibitors, ÿ- blockers, diuretics, spironolactone are used. Although HF in arrhythmogenic cardiomyopathy is due to ventricular systolic dysfunction, cardiac glycosides are contraindicated in these patients due to their proarrhythmic effect.

1.9.5. Unclassifiable cardiomyopathies

Unclassified cardiomyopathy includes various cardiac diseases of unknown nature that do not meet the diagnostic criteria for hypertrophic, dilated, restrictive, and arrhythmogenic cardiomyopathy. The most studied diseases of this

groups are non-compact left ventricular myocardium and takotsubo cardiomyocardium.

Syndrome of non-compact (spongy) myocardium of the left ventricle is a rare type of cardiomyopathy.

The disease is based on a genetically determined disorder of myocardial development in embryonic period. The disease is inherited in an X-linked recessive manner

more common in men. Morphological features of the disease are deep

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trabeculae in the myocardium of the left ventricle and interventricular septum. In some patients, these changes are combined with signs of dilated cardiomyopathy and heart defects: ventricular septal defect, pulmonary artery stenosis. Clinical

manifestations of the disease are heart failure, ventricular arrhythmias, thromboembolic complications. The main diagnostic method is echocardiography. Symptom treatment

tic.

Takotsubo CMP, or stress CMP, is very rare, mostly in postmenopausal women. The first cases of the disease are described by Japanese doctors. Word

"takotsubo" in Japan is called a pot for catching octopuses, which in its shape resembles the spherical dilatation of the left ventricle characteristic of this disease in

top areas. Acute dilatation and decreased contractility of the left ventricle in patients with

Takotsubo CMP is caused by stress, which is why the disease is referred to as "broken heart syndrome". It is believed that the main pathogenetic mechanisms of the disease are a violation of microcirculation in the myocardium, spasm of small coronary

arteries, and a pathological response to hypercatecholaminemia. IMP takotsubo is manifested by a s

HF and/or chest pain associated with anterior ECG findings

THEM. Differential diagnosis is based on a comparison of echocardiography and coronary angiography data, which, as a rule, does not reveal functionally significant

stenosis and thrombosis of the coronary arteries. Inotropic drugs are used to treat patients. facilities. With their insufficient effectiveness, they resort to intra-aortic balloon counterpulsation. In typical cases, the shape and function of the left ventricle are restored within 2 months.

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1.10. TUMORS OF THE HEART

There are primary and secondary (metastatic) tumors of the heart. Primary tumors are divided into benign and malignant. Among benign neoplasms, which make up about 75% of primary tumors of the heart, myxoma is distinguished.

and non-myxomatous tumors (fibromas, lipomas, rhabdomyomas, hemangiomas, neurinomas, etc.).

Malignant neoplasms, accounting for about 25% of primary cardiac tumors,

are represented mainly by various types of sarcomas (rhabdosarcoma, angiosarcoma, liposarcoma, fibrosarcoma, etc.).

Tumors of the heart are rare diseases. Primary tumors are found in 0.8 - 1.2% patients referred to cardiac surgery clinics. According to the pathological

studies, the detection rate of primary heart tumors does not exceed 1.4%.

1.10.1. Myxoma

Myxoma is the most commonly diagnosed benign intracavitary

heart tumor. The frequency of detection of this tumor is about 50% of the total primary neoplasms of the heart. In most cases, cardiac myxoma is diagnosed in

persons 30 - 60 years old. Sporadic myxoma is 3 times more common in women than in men. hereditary myxoma - with the same frequency in both sexes.

Typically, the tumor has a stalk fixed to the interventricular septum. At

In 75% of patients, myxoma is localized in the left atrium, in 20% of patients - in the right atrium. In rare cases, the ventricles of the heart, mitral and tricuspid valves become the location of the tumor. The body of the tumor is spherical, its diameter

varies from 1 to 15 cm. Myxoma has a loose, jelly-like consistency and consists mainly of an amorphous matrix. The tumor tissue contains a small amount

polygonal cellular elements, collagen fibers, small blood vessels,

there are small hemorrhages, foci of necrosis and areas of calcification. On the surface myxomas often form blood clots.

Along with general symptoms (fatigue, weight loss, pain in the

heart of an uncertain nature, arthralgia, fever) frequent clinical manifestations of the tumor are HF, caused by a mechanical obstruction of the movement of blood into

chambers of the heart, and arrhythmias (sinus tachycardia, paroxysms of fibrillation and flutter atrial, ventricular and atrial extrasystoles). Some patients develop

AV block. Often, myxoma of the heart is manifested by thromboembolism and syncopal conditions caused by transient wedging of the tumor body into the valvular (most often left AB) hole.

Auscultatory signs of a tumor, depending on its localization, correspond to mit

ral, tricuspid heart disease, aortic orifice stenosis, or valvular stenosis

pulmonary artery. The severity of auscultatory manifestations of myxoma of the heart depends on body position. A blood test reveals nonspecific changes in the form of leukocytosis, thrombocytopenia, an increase in ESR, the content of C-reactive protein, gamma globulins, interleukin 6. ECG changes are also nonspecific. Some patients show signs of left and/or

right atrial overload, right ventricular hypertrophy.

Chest x-ray may show signs of pulmonary congestion and changes in the boundaries of the heart due to expansion of the left atrium. Two-dimensional echocardiography, magnetic resonance imaging, and computed tomography allow imaging of the tumor.

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