dilated cardiomyopathy. Coding for cardiomyopathy in μb Degrees of compensation for cardiomyopathy

Myocardial dystrophy with menopause.

Myocardial dystrophy in thyrotoxicosis.

Alcoholic myocardial dystrophy.

G62.1 Alcoholic myocardial dystrophy.

Causes of myocardial dystrophy.

The causes are diseases and conditions that lead to depletion, mutation and a decrease in the efficiency of the muscle cells of the heart.

Hypovitaminosis and beriberi (insufficient intake or lack of vitamins in the body).

Myasthenia gravis, myopathy (neuromuscular disorders).

Toxic poisoning (carbon monoxide, barbiturates, alcoholism, drug addiction).

Thyrotoxicosis (thyroid disease).

Anemia (anemia).

Endocrine disorders (violation of protein, fat and carbohydrate metabolism).

Violations of the water and electrolyte balance (dehydration).

Violation of the hormonal background (menopause period).

Pathogenesis and pathological anatomy.

1. Violation of the central regulation leads to an increase in myocardial oxygen demand.
2. Reduced ATP production and oxygen use.
3. Activation of peroxidation, accumulation of free radicals leads to further damage to the myocardium.

Clinical manifestations

Shortness of breath on exertion.

General weakness.

Decreased performance and exercise tolerance.

Heart rhythm disturbances (arrhythmias).

There may be swelling in the legs.

Expanding the boundaries of the heart.

Heart sounds are muffled, systolic murmur at 1 point.

The most common variants of myocardial dystrophy.

Long-term use of alcohol (chronic alcoholism) leads to disruption of cellular structures and metabolic processes in the myocardium.

It develops in women after 45 - 50 years (during menopause or after it).

There is no specific diagnosis of myocardial dystrophy.

The diagnosis is made on the basis of:

clinical symptoms;

ECG signs are an increase in heart rate (tachycardia), arrhythmia and T wave smoothness;

X-ray of the heart: increase in size;

Myocardial biopsy.

Cardiomyopathy (CMP)- a disease of the myocardium, accompanied by its dysfunction.

In 2006, the American Heart Association (AHA - American Heart Association) proposed a new definition of CMP.

Definition of cardiomyopathy

cardiomyopathy- a heterogeneous group of diseases of various etiologies (often genetically determined), accompanied by mechanical and / or electrical dysfunction of the myocardium and disproportionate hypertrophy or dilation.

KMP classification.

Types of cardiomyopathy (WHO, 1995):

Hypertrophic;

dilated;

Arrhythmogenic dysplasia of the right ventricle;

Unclassified.

ANA also proposed a new classification of the ILC:

Primary cardiomyopathy is a disease in which there is an isolated lesion of the myocardium.

Secondary cardiomyopathy is a myocardial lesion that develops with a systemic (multi-organ) disease.

HYPERTROPHIC CARDIOMYOPATHY

DEFINITION

HCM is a hereditary disease characterized by asymmetric hypertrophy of the left ventricular myocardium.

I 42.1. Obstructive hypertrophic cardiomyopathy.

I 42.2. Other hypertrophic cardiomyopathy.

● Non-obstructive.

HCM is an inherited disease that is transmitted as an autosomal dominant trait. Currently, about 200 mutations responsible for the development of the disease have been identified.

chest pain (36-40%);

dizziness, which was regarded as a presyncope state (14–29%);

syncope (36–64%);

weakness (0.4–24%).

PHYSICAL EXAMINATION

Inspection. On examination, there are no characteristic clinical signs.

Palpation. A high, diffuse apex beat is determined, which is often shifted to the left.

The pulse is accelerated.

Auscultation: systolic murmur, which is detected at the apex and in the fourth intercostal space to the left of the sternum.

LABORATORY STUDIES No changes.

DNA analysis of mutant genes is the most accurate method for verifying the diagnosis of HCM.

INSTRUMENTAL STUDIES

From instrumental studies perform:

Electrocardiography (overload and / or hypertrophy of the LV myocardium, rhythm and conduction disturbances),

x-ray examination of the chest: signs of enlargement of the left ventricle and left atrium,

Holter ECG monitoring,

EchoCG is the "gold" standard in the diagnosis of HCM;

Magnetic resonance imaging is indicated for all patients before surgery.

coronary angiography. It is performed with HCM and persistent retrosternal pain (frequent attacks of angina pectoris).

INDICATIONS FOR CONSULTATION OF OTHER SPECIALISTS

To exclude genetic diseases and syndromes, patients should be referred to a genetic counseling specialist.

Consultation with a cardiac surgeon.

DIFFERENTIAL DIAGNOSIS

HCM must be differentiated from diseases accompanied by LV myocardial hypertrophy.

DILATED CARDIOMYOPATHY

DEFINITION

Dilated cardiomyopathy (DCM) is a primary myocardial lesion that develops as a result of various factors (genetic predisposition, chronic viral myocarditis, immune system disorders) and is characterized by a pronounced expansion of the heart chambers.

I 42.0. dilated cardiomyopathy.

EPIDEMIOLOGY

The incidence of DCMP is 5–7.5 cases per 100,000 population per year. In men, the disease occurs 2-3 times more often, especially at the age of 30-50 years.

Cardiomyopathy (I42)

Excludes: cardiomyopathy complicating. pregnancy (O99.4). postpartum period (O90.3) ischemic cardiomyopathy (I25.5)

I42.0 Dilated cardiomyopathy

I42.1 Obstructive hypertrophic cardiomyopathy

Hypertrophic subaortic stenosis

I42.2 Other hypertrophic cardiomyopathy

Non-obstructive hypertrophic cardiomyopathy

I42.3 Endomyocardial (eosinophilic) disease

Endomyocardial (tropical) fibrosis Loeffler's endocarditis

I42.4 Endocardial fibroelastosis

Congenital cardiomyopathy

I42.5 Other restrictive cardiomyopathy

I42.6 Alcoholic cardiomyopathy

If it is necessary to identify the cause, use an additional external cause code (class XX).

I42.8 Other cardiomyopathies

I42.9 Cardiomyopathy, unspecified

Cardiomyopathy (primary) (secondary) NOS

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Among myocardial diseases, there are separately distinguished cardiomyopathy . Cardiomyopathy in adults is the fourth most common cause of chronic heart failure. What is cardiomyopathy? This is a whole group of myocardial diseases that are not inflammatory and non-coronary (not associated with damage to the coronary vessels) in nature. With the disease, there are violations of the structure of the heart muscle and pronounced violations of its function. Cardiomyopathies are often accompanied by myocardial hypertrophy or enlargement of the heart chambers. These diseases develop due to various reasons and often have a genetic nature.

Cardiomyopathy is divided into primary (this includes genetic, without an established cause - idiopathic cardiomyopathy, acquired, mixed) and secondary, developed against the background of various diseases. Primary idiopathic cardiomyopathy can be dilated, hypertrophic, restrictive, arrhythmogenic right ventricular dysplasia ( Fontan's disease ).

The idiopathic form of dilated cardiomyopathy is the most severe: the disease in adults is steadily progressing, and heart failure becomes its debut. Cardiomyopathy of mixed genesis is a pathology of the myocardium, which has the features of a hereditary and acquired disease. Some authors include restrictive cardiomyopathy in this group. Secondary cardiomyopathy includes alcoholic, thyrotoxic, diabetic, dyshormonal, that is, the secondary develops against the background of other diseases.

Pathogenesis

As a result of gene mutations in hypertrophic cardiomyopathy, specific changes occur in the heart muscle - a violation of the orientation of muscle fibers, as a result of which the heart muscle changes and thickens. The pathogenesis is based on an increase in pressure in the left ventricle during diastole (relaxation), as a result of which the ventricle must expel blood with increased force.

An increase in the load on the heart muscle causes an even greater hypertrophy and a decrease in myocardial compliance, as well as a decrease in the cavity of the left ventricle. Exercise and increase outflow tract obstruction and stress on the left ventricle. Pre-syncope states and, characteristic of these patients, occur in connection with a decrease in cerebral circulation and are more often observed during physical exertion, as a result of a small cardiac output due to a reduced ventricular cavity.

The etiology of dilated cardiomyopathy often cannot be established, so it is considered as an idiopathic form. Only 20% of patients associate this disease with infectious transferred earlier. It is believed that damage to the mitochondria of cells, a violation of energy metabolism in the myocardium and a negative effect on myofibrils (a functional unit of a muscle fiber), cause viruses that persist in the body (primarily enteroviruses).

Viral RNA can be included in the genetic apparatus of myocardial cells, and viruses also "trigger" the autoimmune process, which leads to pronounced changes in the structure of myofibrils and disruption of their contractile function. There is also marked dystrophy cardiomyocytes and their necrosis. As a result of such pathological changes, the heart muscle loses its ability to contract.

The pathogenesis of restrictive CMP depends on the underlying disease. In fibroplastic endocarditis, disturbances in the immune response and the release of active substances that damage cardiomyocytes are of primary importance. Subsequently, it develops fibrosis and thickening of the endocardium. Fibrosis entails an increase in myocardial stiffness, as a result of which the diastolic function of the heart . Violation of relaxation reduces the filling period of the ventricles, therefore, the stroke volume decreases and the load on the atria increases (they expand).

In the pathogenesis of heart damage in alcoholic cardiomyopathy, two mechanisms are noted: impregnation of the myocardium with fatty inclusions, which causes degeneration of myocardial cells, and damage to the myocardium and nervous system due to deficiency. Due to the increase in the load on the heart muscle, myocardial hypertrophy develops first, then lipomatosis (impregnation of cells with fat), cell degeneration, expansion of the cavities of the heart, the formation of blood clots inside the cavities and the development heart failure . Alcoholic cardiomyopathy occurs with rhythm disturbance and circulatory failure in both large and small circles. Alcohol intake is a provoking factor of atrial fibrillation.

The genesis of the development of non-compact myocardium is not completely clear. There are several points of view: the violation of embryogenesis and the development of pathology in the process of life. During the period of embryogenesis, the spongy layer of the myocardium normally thickens and a normal “compact” layer is formed - the tubular muscle fibers gradually “grow together”. If this process is disturbed, the myocardium retains a spongy structure, has deep spaces between the trabeculae, which are connected to the LV cavity. If we consider the acquired pathology in the form of a non-compact myocardium, then it can be noted that increased trabecularity of the left ventricular myocardium occurs in athletes, during pregnancy, and in patients with sickle cell anemia. An increase in trabecularity in these cases is considered as an adaptation to physical activity and a response to an increase in the load of the left ventricle.

Classification

Many classifications indicate that this pathology is constantly being studied and improved. Each of these classifications complements one another.

According to the 1995 classification:

• Basic (dilated, hypertrophic and restrictive).
• Additional (arrhythmogenic, postpartum).
• Specific (dysmetabolic, ischemic, inflammatory, hypertensive, with systemic diseases, muscular dystrophy, allergic and toxic).
• Unclassified (fibroelastosis, cardiomyopathy against the background of mitochondriopathies).

The 2006 American Heart Association classification refers to primary cardiomyopathies and subdivides them into:

• Genetic (this includes hypertrophic, arrhythmogenic, mitochondrial myopathies, non-compact myocardial syndrome, glycogenoses that have arisen in violation of the function of ion channels).
• Mixed (restrictive and dilated).
• Acquired (postpartum, IMP takotsubo, with and induced by tachycardia).

• Dilated.
• Hypertrophic.
• Restrictive.
• Arrhythmogenic right ventricle.
• Unclassified (here included non-compact myocardium And KMP takotsubo ).

Hypertrophic cardiomyopathy

It is a genetically determined disease (familial forms dominate), which is recognized at a late stage, since in 50-60% of cases it is detected by chance during echocardiography.

It manifests itself at different ages, depending on the severity of the gene defect. The cause of the disease is mutations in the genes of sarcomeric proteins. Given the genetic factor, the term "familial hypertrophic cardiomyopathy" is often used. The disease is the cause of sudden death of athletes at a young age.

Hypertrophic cardiomyopathy is characterized by severe hypertrophy of the left (more than 1.5 cm) and / or right ventricle, which arose for no reason (there is no previous high blood pressure, the presence of defects and other heart diseases). The interventricular septum, apex and middle segments of the ventricle are involved in the process. The volume of the left ventricle may be normal or reduced. In 25% of patients, there is a systolic pressure gradient in the left ventricle, which is due to obstruction (presence of an obstruction) of the outflow tract of the left ventricle.

Depending on the presence or absence of obstruction, cardiomyopathy has two hemodynamic variants: hypertrophic-obstructive cardiomyopathy and non-obstructive. Hypertrophic - obstructive cardiomyopathy, which implies the obligatory presence of obstruction of the outflow tract.

With this form, several options for obstruction are possible:

• subaortic obstruction is permanent and present at rest, there is a term for this condition "muscular subaortic stenosis";
• labile obstruction - there are significant pressure fluctuations inside the ventricle;
• latent - functional, appears only during exercise or during pharmacological tests.

The presence of obstruction determines the severity of clinical manifestations. Non-obstructive cardiomyopathy proceeds with unexpressed myocardial hypertrophy and clinic.

Dilated cardiomyopathy (DCM)

More common in men. The most common manifestation of DCMP in humans is recorded at 30-45 years of age. Patients with this disease account for up to 55-60% of all patients with CMP. This disease is characterized by the expansion of all chambers of the heart and a pronounced violation of the pumping function of the heart. At the same time, there are no reasons that could cause such violations of the contractile function of the heart (valvular pathology, hypertension , IBS). Violation of contractility causes a decrease in the release of blood into systole, and the residual volume of blood in the ventricles increases. This leads to the expansion of the ventricles and the development of biventricular (left and right ventricular) heart failure.

There are the following forms of dilated cardiomyopathy:

• Idiopathic (develops without an obvious cause and refers to rare diseases).
• Family (hereditary, genetically determined). In 40% of patients, the disease has a family character.
• Viral and autoimmune. It is noted that dilated cardiomyopathy develops against the background of a viral myocarditis. The role of group B is revealed.

A disease of progressive heart failure is manifested. Complicated arrhythmias , thromboembolism that occur at any stage, sudden death is also possible. A feature of chronic heart failure is a severe course and poor prognosis.

Thrombosis and embolism in this form of cardiomyopathy occur 2-3 times more often than in other heart diseases. Parietal thrombi in the left ventricle with echocardiography are found in 40% of cases, the frequency of atrial thrombosis is much higher and depends on atrial fibrillation. Prognostically, recurrent thromboembolism in the pulmonary artery is considered the most dangerous.

The diagnosis of DKMP is established on the basis of:

• X-ray examination, which indicates an increase in the heart, increased intraventricular pressure (it is evidenced by the spherical shape of the heart), signs of venous congestion.
• Ultrasound examination of the heart: dilatation of the cavities, normal or slightly altered wall thickness, decreased ejection fraction and contractile function of the myocardium, insufficiency of atrioventricular valves, the presence of blood clots in the heart cavities.

Code for dilated cardiomyopathy according to ICD-10-I42.0.

Restrictive cardiomyopathy

A rare lesion of the heart muscle, which leads to a violation of the filling of the ventricles (one or two) during diastole and a decrease in the volume of their cavities. The atrial cavities are enlarged. At the same time, there are no changes in the thickness of the myocardium. In the wall of the myocardium there is a massive fibrosis (they become rigid), and all chambers of the heart are involved in the process. In this regard, restrictive cardiomyopathy includes several different diseases that are characterized by the development of fibrosis:

• endomyocardial fibrosis ;
• fibroplastic Loeffler's endocarditis ;
• fibroelastosis of newborns;
• African obliterans KMP.

Restrictive cardiomyopathy may be associated with collagenosis, neoplastic processes and the use of anticancer drugs, the consequences of local. In cases where restrictive cardiomyopathy is formed without pathology of the heart valves, pericardium and epicardium, the diagnosis is "idiopathic restrictive cardiomyopathy". The disease is either asymptomatic or disturbed by asthma attacks, loss of consciousness during physical exertion, and frequent pulmonary infections.

This is due to the fact that in some patients diastolic dysfunction appears early and is pronounced, so complaints and signs of heart failure appear early. In other cases, diastolic dysfunction has no clinical manifestations, so the condition of patients does not worsen for many years.

Non-compact myocardium, or rather non-compact left ventricular syndrome, as it is more often affected, and takotsubo cardiomyopathy .

Non-compact cardiomyopathy of the left ventricle is a heterogeneous (the same disease is caused by mutations in different genes) disease that is characterized by an abnormal two-layer structure of the myocardium. The first layer is represented by a compact myocardium, normal in structure, and the second has a spongy structure with trabeculae and spaces between them, which communicate with the cavity of the left ventricle (it is affected more often).

The ratio of non-compact and compact layer is 2:1. That is, the non-compact myocardium is wide, and the layer of the true myocardium is thin and practically non-functional. This structure of the myocardium causes a deterioration in the contractile function of the ventricle, which progresses and rapidly develops fatal heart failure, ventricular arrhythmias and systemic thromboembolism. With the introduction of genetic research methods, the possibility of early diagnosis has become possible. In 50% of patients, this pathology is detected in the family. Mutations in sarcomere genes are more often observed - such mutations are found in both dilated and hypertrophic cardiomyopathy.

Takotsubo cardiomyopathy

The basis of takotsubo cardiomyopathy (or stressful cardiomyopathy, "broken heart syndrome") is acute inflammation of the myocardium, which arose against the background of a massive ejection catecholamines ( , And ). The disease occurs more often in women after severe stress. At the same time, acutely developing LV systolic dysfunction (impaired contractility) is formed.

In typical cases, patients experience (myocardial infarction): intense pain in the chest, severe tachycardia, signs of a heart attack on the electrocardiogram, but the symptoms may resolve in a few days. Many have complications: pulmonary edema, arrhythmias, rupture of the LV wall, arterial hypotension and death. Complications in the form of strokes (ischemic or hemorrhagic) are also possible. A frequent complication of the early period is shock.

Specific cardiomyopathies include dysmetabolic (metabolic), ischemic, valvular, hypertensive, allergic/toxic (alcohol, drug, radiation), cardiomyopathy in collagenoses, and muscular dystrophy.

Ischemic cardiomyopathy

This type of CMP is considered as a form of coronary heart disease. Cardiomyopathy of this type occurs against the background of widespread and severe atherosclerosis of the main, coronary arteries and myocardial infarction. The pathological state of the myocardium develops as a result of constant attacks myocardial ischemia . Frequent and prolonged episodes of ischemia cause degenerative changes in myocytes and their death.

Ischemic cardiomyopathy occurs at the age of 45-55 years, mainly among men. In the formation of this cardiomyopathy, an important role is played by postinfarction remodeling (change) of the myocardium and its hibernation. Hibernation is an adaptive process of reducing the contractile function of the heart in proportion to the decrease in blood flow. This helps to prevent the further development of ischemia in the myocardium and the development acute myocardial infarction . Reduced contractile function is completely normalized when normal blood supply is restored.

Ischemic cardiomyopathy occurs with the expansion of the cavities of the heart (first of the left ventricle, then other chambers), thinning of the walls of the left ventricle and significant cardiomegaly, all this leads to a pronounced violation of the pumping function of the heart and chronic heart failure.

Ventricular remodeling occurs slowly with the inclusion of compensatory mechanisms, in which the chambers of the heart gradually increase. Heart failure also progresses slowly, starting with the left ventricular, and in the later stages, the right ventricular also joins.

The characteristic signs of this cardiomyopathy are an increase in the mass of the heart, biventricular expansion, severe atherosclerotic lesions of the coronary arteries, and disproportionate thinning of the walls of the ventricle (the thickness of unchanged muscle tissue is not more than 11 mm). The diagnosis of CMP indicates a long history and widespread damage to the coronary vessels, so the main method of research is coronary angiography .

Metabolic cardiomyopathy

Metabolic cardiomyopathy, what is it? This group of cardiomyopathies combines diseases of the heart muscle that have arisen against the background of a metabolic disorder in the body. A synonym is dysmetabolic cardiomyopathy and belongs to the class of dilated cardiomyopathy. ICD-10-I43.1 code for dysmetabolic cardiomyopathy (cardiomyopathy with metabolic disorders). Perhaps this is the largest group of secondary myocardial lesions, since it is caused by various and numerous reasons:

• Endocrine pathology (, pheochromocytoma , hypothyroidism , diabetes , ).
• Hereditary storage diseases: Neman-Pick disease , Hurlera , Fabry , hemochromatosis , storage diseases glycogen .
• Deficiency of electrolytes (potassium, magnesium, selenium, iron, B vitamins).
• Amyloidosis - complex metabolic changes, as a result of which the deposition of proteins (amyloids) in tissues is noted.
• Intoxication (drug, alcohol, endotoxicosis in various diseases).
• Hormonal changes (natural and artificial).
• Negative effect of the environment (radiation, hypothermia, etc.).
• stressful situations.

Of the secondary cardiomyopathies that have arisen against the background of endocrine pathology, the following should be distinguished:

• thyrotoxic cardiomyopathy. Against the background of severe uncompensated thyrotoxicosis with diffuse toxic goiter, dilated cardiomyopathy develops, valve insufficiency (mitral and tricuspid), atrial fibrillation and circulatory failure. Cardiomyopathy goes through three stages: myocardial dystrophy and hypertrophy of the heart muscle (they are reversible if treated correctly); compensatory hypertrophy and expansion of cavities (changes are partially reversible); myocardiosclerosis , which indicates irreversible changes and leads to the development of heart failure. The task of treating thyrotoxicosis is to achieve a euthyroid state, for which thyreostatics are prescribed, which block the synthesis of hormones by the gland. When treated with thyreostatics, signs of circulatory failure in DCM at rest are eliminated, atrial fibrillation disappears, ejection fraction increases, and even the size of the heart cavities is normalized. In parallel appointed b-blockers .
• Acromegaly is characterized by excessive production of growth hormone and IGF-1, which actively affect cardiomyocytes. Among the cardiovascular disorders in acromegaly, there is myocardial hypertrophy , interstitial fibrosis And acromegalic cardiomyopathy , the main risk factor of which is the duration of the disease.

Dishormonal cardiomyopathy (climacteric)

Dishormonal cardiomyopathy is associated with impaired activity estrogen , which favorably affect the electrolyte and protein metabolism in the myocardium and regulate the effect of the sympathetic nervous system on the heart. The disease develops with estrogen deficiency in menopause and in young women with gynecological diseases (,), with post-castration premenstrual syndrome. With pathological menopause, metabolic disorders develop in the heart muscle, which lead to dystrophic changes, in most cases reversible. Only some women run out myocardiofibrosis . This form of cardiomyopathy can occur acutely and develop gradually.

Most often, women complain of prolonged pain in the region of the heart, which has a diverse character. A distinctive feature is that cardialgia is not provoked by physical activity, can last for several days and is not eliminated by taking. Women develop autonomic disorders: palpitations, dizziness, dissatisfaction with inhalation and "lack of air" even at rest, hot flashes. Pressure fluctuations and general neuroticism are characteristic.

With menopausal IMP, there may be the following rhythm disturbances:

• ventricular extrasystole ;
• sinus arrhythmia ;
• supraventricular tachycardia .

The examination reveals a discrepancy - a lot of cardiac complaints in the absence of signs of coronary or heart failure.

Toxic cardiomyopathy

Toxic CMP is caused by exposure to drugs, toxic substances at work, and alcohol abuse. It was found that the majority of patients who in their professional activities were exposed to constant and prolonged exposure xenobiotics (chemical substances alien to the body), cardiomyopathy developed, which had the character of dilatation.

Under the influence of xenobiotics, cardiomyocytes develop dystrophy and necrosis of some cells, and, as a result, compensatory hypertrophy of others. In the interstitium of the myocardium, there were: edema And fibrosis marked sclerosis of the endocardium. All these processes lead to wall hypertrophy and dilatation of the heart chambers. Often, parietal blood clots are found in the chambers of the heart, which caused death. At the same time, patients do not have changes in the coronary vessels.

Toxic cardiomyopathy also develops against the background of endotoxicosis, for example uremic cardiomyopathy . With uremia parathyroid hormone is released in elevated concentrations and becomes the "universal uremic toxin". At the same time, tissue metabolism is disturbed very early, and the products of impaired protein and lipid metabolism secondarily damage the heart muscle. It changes the content of protein and lipids, the activity of tissue enzymes decreases, changes occur in DNA and RNA. Toxic products also cause vascular disorders, which further support the processes of damage to cardiomyocytes. In chronic endotoxicosis, changes in the heart muscle are types of dysmetabolic cardiomyopathy.

An example of the effects of drugs is neuroleptic cardiomyopathy. Taking cardiotoxic phenothiazine neuroleptics (, ) leads to secondary toxic (metabolic) cardiomyopathies, which in form are dilated. There is diffuse myocardial damage, a pronounced decrease in its contractile function and progressive CHF.

is a disease characterized by a combination of mental and somatic disorders. Most often, alcoholism affects the liver and heart. Alcoholic cardiomyopathy refers to secondary toxic dilated cardiomyopathy. In ICD-10, alcoholic KMP is separated into a separate form - I 42.6. The disease develops with alcohol abuse for 10 years. Recently, there has been a clear trend towards a reduction in this period. Severe heart failure that developed against the background of CMP, thromboembolism and rhythm disturbances often cause death.

Some cardiomyopathies (dilated and familial forms of cardiomyopathies) are characterized by cardiomegaly which means "big heart". The heart increases in size due to hypertrophy of the heart muscle and expansion of the cavities. Often, the metaphorical term "bull's heart" is used to define this condition, which only reflects the visual appearance of the organ. The term “bovine heart disease” is not entirely correct, since such a disease does not exist. This is a syndrome that is observed in various diseases and conditions:

• congenital heart defects;
• systemic collagenoses ;
• viral myocarditis,
• polycythemia ;
• arteriovenous anastomoses;
• erythroderma ;
• Paget's disease ;
• abuse of beer.

Cardiomegaly can also develop while taking certain medications ( Daunorubicin , Emetine , tricyclic antidepressants, phenothiazine antipsychotics, sulfa drugs). For various reasons, an increase in the heart is compensatory - it works in an enhanced mode, pumping a larger volume of blood and increases, and the chambers expand.

For example, with aortic defects in the heart, a pronounced working hypertrophy of the left ventricle occurs. It can be the size of a human head. The mass of the heart in aortic valve insufficiency can reach 900 g or more. Despite the fact that the size of the organ is increased, the volume of the functioning mass is significantly reduced. Muscle cells are replaced by connective tissue, the myocardium becomes flabby and weak, while the heart reduces its pumping function and heart failure develops.

Causes

The causes of diseases are different and among the main ones should be highlighted:

• genetic factors. For example, 200 mutations of several genes encoding myofibril proteins have been described in hypertrophic cardiomyopathy.
• Hereditary storage diseases.
• Viral diseases.
• Endocrine pathology.
• electrolyte deficiency.
• Amyloidosis .
• Toxic effects (alcohol, pesticides, xenobiotics, excessive intake of cobalt).
• Endotoxicosis .
• Taking medications (anticancer, neuroleptics, sulfonamides
• Hormonal changes.
• Environmental impact (including radiation exposure).
• stressful situations.

Symptoms of cardiomyopathy

The clinical picture of all cardiomyopathies includes:

• Signs of heart failure.
• Rhythm and conduction disturbances.
• thromboembolic complications.

Some types have distinctive features of the clinic inherent only to them.

Symptoms of hypertrophic cardiomyopathy

Patients are concerned about fatigue, shortness of breath on exertion and at rest, and nocturnal attacks. cardiac asthma . Pain in the region of the heart are anginal in nature - compressive-pressing behind the sternum that occurs after physical exertion. They last longer than angina pectoris .

Pain in the heart is due to a decrease in oxygen delivery with an increased need for oxygen. A specific symptom is pre-syncope and fainting, which are observed in 77% of patients. They are caused by arrhythmias (ventricular extrasystoles are characteristic, which are the earliest symptom), severe hypotension and impaired blood supply to the brain. The frequency of syncope varies significantly in different patients: from daily and frequent to single, appearing every few months. Syncope is more common in patients with outflow tract obstruction.

There are several variants of the course of the disease:

• Stable and good quality.
• Progressive - over time, all symptoms become pronounced in view of the progression of heart failure.
• Sudden death.
• The development of atrial fibrillation and complications in the form of thromboembolism.

Manifestations of dilated cardiomyopathy

Complaints of patients are non-specific and are associated with heart failure: weakness, fatigue, shortness of breath, dizziness, swelling of the legs, cough, abdominal enlargement (ascites), palpitations and arrhythmias. Combination of ventricular and supraventricular extrasystoles , which go into atrial fibrillation . Thromboembolism also develops, the source of which is blood clots in the dilated cavities of the heart. Often, dilated cardiomyopathy appears acutely and suddenly without cause or after a viral infection, pneumonia, or childbirth. These patients develop left ventricular and right ventricular failure.

Symptoms of Restrictive Cardiomyopathy

The early stages are characterized by the appearance of weakness and paroxysmal shortness of breath at night. In the later stages, congestive heart failure develops with liver enlargement, ascites, and swelling of the neck veins. Patients complain of severe shortness of breath at the slightest load, heaviness in the right hypochondrium, swelling, and an increase in the abdomen. The condition of patients is severe and there is a discrepancy between the severity of heart failure and the absence of heart enlargement. An increase in the atrial cavities creates conditions for the appearance of supraventricular arrhythmias: atrial arrhythmias and atrial fibrillation in combination with blockade.

Symptoms of alcoholic cardiomyopathy

Symptoms are non-specific and indicate chronic heart failure: shortness of breath, congestion in the lungs, edema, varicose veins of the neck. Often noted arrhythmias , less often thromboembolism . Heart failure is the cause of death in 3% of cases. In the clinical picture of these patients, the foreground is liver failure , polyneuropathy due to a significant deficit.

Tests and diagnosis of cardiomyopathy

Diagnosis of any form of cardiomyopathy includes:

• echocardiographic study.
• X-ray examination.
• Holter ECG monitoring.

Diagnosis of hypertrophic cardiomyopathy

Based on echocardiography, but the "gold" standard of diagnosis is the detection of gene mutations, since individual mutations are associated with a poor prognosis and a high risk of sudden death. With a mutation in the gene troponin T the patient develops moderate hypertrophy, but the prognosis is unfavorable and there is a high probability of sudden death. Other genetic mutations are accompanied by a more favorable course and prognosis.

Classic echocardiographic features of HCM:

• Asymmetric ventricular septal hypertrophy (usually greater than 13 mm). The hypertrophic area is found on the septum and free wall. Less often, only one septum is affected.
• Hypokinesia hypertrophied area (usually interventricular septum).
• Small size and volume of the left ventricle.
• Intraventricular pressure gradient greater than 30 mm Hg. Art. at rest (with a load - more than 50 mm Hg. Art.).
• Mitral valve prolapse and mitral insufficiency.
• Systolic movement of the mitral valve.
• Systolic occlusion of the aortic valve.

On the electrocardiogram with HCM - signs of left ventricular hypertrophy, deep Q waves, ST segment elevation, in the left chest leads - negative T waves. The last sign may indicate myocardial hypertrophy in the apex. With Holter monitoring, arrhythmias are determined (more often ventricular and atrial).

Examinations for DCMP

X-ray examination is the first step that allows you to suspect this pathology. An increase in the heart, signs of increased pressure inside the ventricles and signs of venous congestion are detected. Echocardiography determines the expansion of the cavities of the heart with an almost unchanged wall thickness, a decrease in contractility and ejection fraction, insufficiency of the atrioventricular valves, blood clots in the ventricular cavity. To confirm the diagnosis, stress ECHO-KG is performed with dobutamine , if it is needed - endomyocardial biopsy And angiography .

Diagnosis of restrictive cardiomyopathy

On echocardiography:

• there is no dilatation and hypertrophy of the ventricles;
• the contractile function of the heart is not disturbed;
• ventricular cavities may decrease;
• the presence of parietal thrombi;
• insufficiency of the bicuspid and tricuspid valves is determined due to an increase in the atria;
• in the study of the transmitral flow, the restrictive type of diastolic function is determined.

Treatment of cardiomyopathy

The treatment of different types of cardiomyopathies is different. But in general, it is aimed at reducing the manifestations of heart failure, eliminating arrhythmias and preventing thromboembolic complications.

Treatment of dilated cardiomyopathy

It should be noted that the treatment of dilated cardiomyopathy with heart failure involves lifelong use of angiotensin-converting enzyme inhibitors ( , Ramipril , ) and β-blockers, the abolition of which is accompanied by a deterioration in the patient's condition. Moreover, the appointment of the first group of drugs provides an improvement in the prognosis of the disease. According to indications, diuretics, cardiac glycosides and peripheral vasodilators are prescribed - they do not affect the prognosis, but positively affect the symptoms of the disease.

The following β-blockers have been proven effective in the treatment of heart failure:,. With severe heart failure, it is prescribed, which affects the reduction in mortality of patients.

Diuretics are prescribed only for fluid retention, as they have side effects that adversely affect the course heart failure . In congestive heart failure, diuretics are prescribed in two stages: active diuretic therapy, which eliminates fluid retention, edema, congestion in the lungs, ascites and maintenance therapy - daily intake of the minimum dose.

According to modern standards, cardiac glycosides are taken for a long time in small doses - 0.125-0.25 mg per day. Higher dosages have been shown to increase the risk of arrhythmias. With atrial fibrillation, the dose is increased to 0.5 mg. Digoxin is prescribed along with diuretics, β-blockers and ACE inhibitors.

Given the frequent development of thromboembolism, anticoagulant therapy is an important direction of treatment. Indirect anticoagulants () are most commonly used.

There is evidence that growth hormone supplementation improves cardiac function and has an anti-inflammatory effect in idiopathic dilated cardiomyopathy. In one study, the drug was administered at 4 IU every other day for three months and these results were obtained. Maybe, a growth hormone will be recommended in the form of immunomodulatory therapy to improve hemodynamics.

β-blockers remain the most effective drugs used in the treatment of hypertrophic cardiomyopathy, as they effectively eliminate shortness of breath, palpitations, angina pectoris, as they reduce the oxygen demand of the heart muscle. Since this group of drugs suppresses the activity of the sympathoadrenal system, an increase in the subaortic pressure gradient is prevented in latent and labile obstruction. By reducing heart rate and preventing ischemia, β-blockers improve LV filling. There is evidence that this group of drugs inhibits the development of hypertrophy and even leads to its reverse development.

Of the β-blockers in this form of cardiomyopathy, preference is given to or. They are prescribed 20 mg three times a day, increasing the dose to the maximum (sometimes even 120-240 mg / day). The lack of effect from taking β-blockers is associated with insufficient dosage. There is an opinion that cardioselective β-blockers ( , ) do not have advantages over non-selective ones, since they must be taken in high doses and selectivity is almost lost.

In severe supraventricular and ventricular arrhythmias, a drug is prescribed (combines the action of a β-blocker and antiarrhythmic).

The use of calcium channel blockers eliminates asynchronous myocardial contraction, stops the development of hypertrophy, and improves myocardial relaxation. Among calcium channel blockers, preference is given to ( Isoptin , ). It increases exercise tolerance and reduces the subaortic pressure gradient.

Since activation of the renin-angiotensin systems is noted in patients, in this regard, it is advisable to use ACE inhibitors in patients with a non-obstructive form and symmetrical myocardial hypertrophy. But drugs of this group are not prescribed for obstructive form, since they can increase obstruction. With hypertrophic cardiomyopathy, nitrates and cardiac glycosides are not prescribed, since they increase the obstruction.

Features of the treatment of KMP takotsubo

Given the fact that Takotsubo CMP is an acute condition, the following approaches are used in treatment:

• anticoagulants (heparin) are administered to reduce thrombosis;
• continuous monitoring of cardiac arrhythmias;
• excluded from treatment catecholamines And organic nitrates ;
• angiotensin-converting enzyme inhibitors are prescribed - they, with prolonged use, reduce the risk of recurrent attacks.

The doctors

Medications

• β-blockers:,.
• Calcium channel blockers: Cardil .
• Angiotensin converting enzyme inhibitors: Fasinopril .
• Antiarrhythmic:,.
• Cardiac glycosides: .
• Peripheral vasodilators: Naniprus , Apressin .
• Anticoagulants:,.

Procedures and operations

Of the surgical methods for the treatment of hypertrophic cardiomyopathy, one can name:

• implantation of a defibrillator (at high risk of sudden death);
• dual-chamber synchronized AV stimulation (implantation of a pacemaker in case of conduction disorders);
• myotomy /myectomy (reduction of the thickness of the hypertrophied myocardium by surgery);
• obstruction of the interventricular artery by introducing ethyl alcohol into it; as a result of the procedure, necrosis with scarring develops in the area of ​​the myocardium and interventricular septum, which helps to reduce the thickness of the area;
• heart transplantation (with a significant decrease in the ventricular cavity, severe hypertrophy and the development of severe heart failure).

Heart transplantation is a radical method for the treatment of end-stage heart failure, which cannot be corrected either by medication or by surgery. The main indication for its implementation is severe circulatory failure, which develops with dilated cardiomyopathy.

An organ transplant operation is associated with various complications in the post-transplant period:

• Postoperative infection, which is in the first place among the complications. Among viruses, the infectious process is most often caused by herpes simplex virus And cytomegalovirus . It is believed that cytomegalovirus plays a role in the development of the rejection reaction and pathology of the coronary arteries. For prevention, antiviral treatment is carried out for a long time. Candidiasis and aspergillosis cause severe pneumonia.
• Arterial hypertension .
• Pathology of the coronary arteries. It is the leading cause of death after surgery. The manifestations of this pathology are painless ischemia and sudden death.
• Chronic rejection of a donor organ.

In children

If in adults the main causes of chronic heart failure are hypertension and coronary artery disease, then in childhood it most often develops with cardiomyopathy. Among cardiomyopathies, the most common is dilated (80.2%), less often - restrictive cardiomyopathy. Cardiomyopathy in children is a serious disease, continuously progressing and with high mortality. In newborns and children under six months, the cause of sudden death is congenital heart defects, and in the second year of life - cardiomyopathy .

Dilated cardiomyopathy characterized by an increase in cavities more often than the left sections and there is no myocardial hypertrophy. The child has a decrease in cardiac output due to a significant decrease in the contractile function of the myocardium. It occurs in children of all ages. In patients at an early age (often after acute respiratory viral infections), the course is acute with symptoms of left ventricular heart failure and the presence of hydropericardium , which is determined by echocardiography. In older children, it develops for no apparent reason and gradually proceeds with biventricular heart failure, the presence of arrhythmias and thromboembolism.

With dilated cardiomyopathy in childhood, course options are possible: favorable, slowly progressive (most often), rapidly progressive and recurrent (also quite often). A favorable course occurs in children who fell ill before 2 years of age, and a progressive course occurs in those who fell ill at an older age.

Treatment of children with chronic heart failure includes beta-blockers, ACE inhibitors, diuretics, and, if indicated, cardiac glycosides. Beta-blockers are not recommended for patients in a state of decompensation, they are added to the treatment after the elimination of edema. At hypotension are included in the complex of treatment. Children with cardiomyopathies should be constantly monitored and periodically hospitalized for correction of treatment, taking into account the changed manifestations of the disease.

The causes of cardiomyopathy in children are different:

• Gene or chromosomal disorders that lead to metabolic disorders. In children, more often than in adults, the cause of this disease is genetically determined congenital metabolic defects.
• Viral infection transmitted during (for example, rubella) or by a child.
• Impact of negative environmental factors.
• Use of teratotoxic drugs or drugs during pregnancy. The impact of negative factors during the laying of the cardiovascular system (the second to eighth weeks of pregnancy) adversely affects the formation of the heart.

Genetic testing is of great importance. For example, it is important to identify mutations in the genes responsible for the development of hypertrophic cardiomyopathy or mutations in the gene GLA(Fabry disease).

Cardiovascular lesions in Fabry disease are manifested by hypertrophic cardiomyopathy (primary left ventricular hypertrophy), arrhythmias, and heart failure. This is a hereditary disease that belongs to the group of lysosomal storage diseases. Characterized by the absence of an enzyme α-galactosidase A or decrease in activity.

Enzyme deficiency leads to the accumulation of glycophospholipids in the lysosomes of the heart, vascular endothelium, kidneys and nervous system. The disease is manifested by pain in the limbs (hands and feet), weakness, the appearance of angiokeratoma (vascular formations of the skin), damage to the heart and kidneys, possible ischemic attacks And . The standard for diagnosing this disease is to determine the activity of the enzyme α-galactosidase A. Its activity is reduced in plasma, leukocytes, in any biopsy or lacrimal fluid. Diagnosis can be made prenatally. The activity of the enzyme is being studied αGAL in fetal plasma or villus culture chorion or amnion .

Gurler syndrome just like Fabry's disease is a storage disease. This is a severe form of mucopolysaccharidosis type 1. This syndrome is associated with mutations in the gene IDUA, which leads to the absence of the alpha-L-iduronidase enzyme, as a result of which dermatan sulfate and heparan sulfate accumulate in the lysosomes. This syndrome is characterized by skeletal deformities, heart defects, liver enlargement, respiratory and cognitive function disorders, and a characteristic appearance of the child.

Genetic research is available. Prenatal diagnosis is possible - enzymatic activity is measured in chorionic villi or amniocytes. Patients are recommended hematopoietic stem cell transplantation and lifelong enzyme replacement therapy laronidase .

Pompe disease also applies to storage diseases. It develops in connection with a metabolic disorder, which is caused by a deficiency of the acid alpha-glucosidase enzyme (it is necessary for the metabolism of glycogen in muscles, heart and liver). The accumulation of unmetabolized glycogen in the tissues causes the characteristic manifestations of the disease - progressive muscle weakness ("sluggish baby" syndrome, feeding difficulties, increased body flexibility and inability to hold the head).

The infantile form of Pompe disease appears in the neonatal period and has a rapidly progressive course. It is with this form of the disease that a pronounced cardiomegaly And cardiomyopathy . Unfortunately, heart lesions are diagnosed at the age of 4 months in 92% of babies. This neuromuscular disease can be treated, but the effectiveness of treatment depends on the timing of the start of therapy. The only means of pathogenetic therapy of this disease is the drug Myozyme .

Endocardial fibroelastosis - a rare heart disease in children that begins in utero and is fully manifested in infants. This disease is characterized by thickening of the endocardium (the inner lining of the chambers of the heart) of several chambers, enlargement of the heart, and early development of heart failure. Primary fibroelastosis is associated with a mutation of a certain gene, and the secondary one develops against the background of obstructive heart defects (often its left sections). In any case, this is the reaction of the endocardium to cardiac stress, which results in the transition of normal endocardial cells into fibroblasts that produce collagen And elastin .

The result of these processes is widespread thickening of the endocardium. At primary fibroelastosis of the endocardium the heart increases significantly in size and has the shape of a ball. The process of fibrosis extends to the leaflets of the mitral and aortic valves with impaired function and with an increased risk of thrombosis. In one variant of the course, the disease manifests itself already in the first hours after birth with acute heart failure, cardiogenic shock and thromboembolism . The child's condition is extremely serious and cannot be saved (the child dies within a week). In the second variant of the course, the disease manifests itself by 3-6 months. Perinatal ultrasound diagnosis of this disease is possible from the second trimester.

Many hereditary or congenital diseases can be detected during pregnancy. So cardiomegaly in the fetus, detected by ultrasound, indicates various heart defects or cardiomyopathy, which has a different genesis. Pathology in the form of an enlarged heart is detected at the second screening (at 16-20 weeks). Of course, the diagnosis of congenital heart defects in the fetus is the most difficult task of perinatology, and their detection in early pregnancy is effective only in 40%.

However, it is possible to identify cardiomegaly in the future to carefully monitor the development of the fetus, to make additional examination and conclusions about the advisability of maintaining pregnancy in a pathology that is incompatible with life.

Cardiomegaly is one of the ultrasound signs of fetal heart failure, it is also detected against the background of:

• antenatal distress (mainly dilated cardiomegaly);
• intrauterine carditis (hypertrophic);
• idiopathic myocardiopathy (dilated).

Dilatation of only the right parts of the heart is possible due to venous congestion with aneurysm of the vein of Galen (arteriovenous malformation of the vein of Galen). This is a rare congenital pathology. In the third trimester of pregnancy, prenatal diagnosis of an aneurysm of the vein of Galen is performed (ultrasound and magnetic resonance imaging of the fetus). When diagnosing, the size of the formation, the presence of cardiomegaly, heart failure and hydrocephalus . This makes it possible to predict delivery and subsequent treatment of the child. Treatment (surgical) should be carried out until irreversible cardiac and neurological complications occur.

In addition to cardiomyopathy, ultrasound signs of fetal heart failure include:

• Reverse blood flow in the venous duct.
• Pulsation of the vein of the umbilical cord.
• Expansion of the right parts of the heart.
• Tricuspid and mitral (blood return).
• Dropsy .

To clarify the diagnosis, fetal echocardiography And doppler echocardiography . In 40% of cases, cardiomegaly is detected, which is not combined with congenital heart defects.

• Often, with hemolytic disease of the fetus and general dropsy, cardiomegaly is also detected. The first signs of hemolytic disease are detected at the 18-20th week of pregnancy.
• Cardiomegaly is registered with an infectious lesion of the fetus and rubella virus . With intrauterine rubella syndrome, stenosis of the pulmonary trunk and coarctation of the aorta can be additionally determined.
• Dilated cardiomegaly develops against the backdrop fetal anemia .
• Hypertrophic cardiomyopathy and fetal cardiomegaly are associated with diabetes in a pregnant woman. Chronic hyperglycemia in the second half of pregnancy causes severe in the fetus. Mothers with insulin-dependent diabetes mellitus give birth to children with signs of diabetic macrosomia (weight over 4000 g), 50% of such newborns have diabetic cardiomegaly and cardiomyopathy. This pathology complicates adaptation in the early neonatal period.
• Hypertrophic cardiomegaly of the recipient fetus develops with monochorionic twins. The so-called fetofetal transfusion syndrome is due to the presence of vascular messages and shunting of blood from one fetus to another. In this case, one fetus is a donor, and the other is a recipient. The former develops anemia, developmental delay, and oligohydramnios, while the latter develops cardiomegaly, erythremia (high red blood cells), dropsy, and polyhydramnios.

If ultrasound markers of chromosomal pathology or malformations are detected, cordocentesis is performed - a method of prenatal diagnosis of hereditary diseases. For this study, blood is obtained from the umbilical cord of the fetus. The optimal study period is 16-20 weeks (in Russia it is carried out at 20-21 weeks).

Some medical genetic centers conduct non-invasive prenatal diagnosis of fetal chromosomal pathologies, which is an alternative cordocentesis . The presence of non-cellular fetal DNA in the mother's blood makes it possible to conduct non-invasive prenatal studies for the fetus.

There is an assumption that in the pathogenesis of idiopathic cardiomyopathies is important mitochondrial dysfunction . Mitochondrial diseases are caused by inherited mutations in mitochondrial DNA . Cardiomyopathies have been described in mitochondrial diseases such as MERRF, MELAS, Barth and Kearns-Sayre syndrome. A distinctive feature of diseases associated with mutations in mitochondrial DNA is their relatively late onset. These diseases are passed on through the mother.

Barth syndrome causes the death of male fetuses (miscarriages or stillbirths). Women are carriers of the mutant gene and have a 50% chance of passing the gene on to their sons. Girls born from female carriers are healthy. Cardiomyopathy (a combination of dilated and hypertrophic) is the main symptom of Barth's syndrome and in 70% of cases it develops already in the first year of life. Patients are at risk of ventricular arrhythmias and death. In some patients, conservative treatment of heart failure is ineffective, which requires heart transplantation at the age of one to 15 years.

Kearns-Sayre syndrome manifested by neurological disorders (convulsions, muscle spasticity, cerebellar ataxia, sensorineural hearing loss). On the part of the endocrine system, insufficient growth and diabetes . From the side of the heart cardiomyopathy and conduction disturbance.

A genetic examination of mitochondrial DNA is carried out in a woman to clarify the diagnosis, which reduces the risk of giving birth to a sick child. There are "mitochondrial panels" that include studies of genes responsible for mitochondrial diseases. Mitochondrial DNA is found in all cells, so bones, teeth, fragments of body parts, blood, semen, hair or nails are suitable for obtaining it.

Diet

For all cardiomyopathies complicated by chronic heart failure, a salt-restricted diet (heart failure diet) should be followed. In general, nutrition depends on the underlying disease. With obesity, restriction of fats and carbohydrates is indicated (diet for obesity), with hypercholesterolemia - restriction of animal fats (diet for vascular atherosclerosis). If the patient has diabetes, against which cardiomyopathy has developed, the diet should be appropriate (diet for diabetes), but with salt restriction.

Prevention

Primary prevention of myocardial lesions with unknown etiology is not carried out. For specific secondary cardiomyopathies, treatment of the underlying disease is important. Prenatal diagnosis (screening for gene mutations, fetal echocardiography) makes it possible to prevent the birth of children with severe congenital heart disease incompatible with life.

General measures to prevent exacerbation and progression of existing myocardial lesions include:

• Exclusion of excessive physical exertion (sports), which cause aggravation of myocardial hypertrophy and sudden death. At the same time, patients are shown the load according to their capabilities.
• Prevention of viral diseases that aggravate the course of cardiomyopathy.
• Work in protective equipment in contact with harmful factors at work.
• Exclusion of factors contributing to the progression of the disease.
• Implementation of the necessary dietary recommendations, the main of which is the restriction of salt intake.
• Weight loss at .
• Refusal of alcohol and smoking.

Consequences and complications

• Severe forms of arrhythmias.
• Heart failure .
• Thromboembolism .
• Sudden cardiac death.

Forecast

The prognosis for restrictive cardiomyopathy depends on the underlying cause of its development, but is generally unfavorable - 70% of patients die within five years. Dilated cardiomyopathy has a severe course and an extremely poor prognosis - a two-year survival rate of DCM in the family form is observed in only 34%-36% of patients. With DKMP of a viral / immune nature - 50%.

If heart failure has already developed with alcoholic cardiomyopathy and the patient does not stop drinking alcohol, the prognosis is unfavorable: death from alcoholic cardiomyopathy occurs in 75% of cases within 3 years. Quitting alcohol before the onset of severe heart failure can halt the progression of ACM or even reverse it.

In the hypertrophic form, two options are possible, depending on gene mutations. The average life expectancy in hypertrophic cardiomyopathy also depends on this. In the presence of malignant mutations with high gene penetrance (frequency of gene manifestation in signs), degree of hypertrophy, severe course and complications, high risk of death, life expectancy is up to 30-35 years.

With gene mutations troponin T mortality is high (up to 90%) even in the absence of myocardial hypertrophy. With benign mutations, there is a low gene penetrance, an unexpressed clinic, a low risk of death, and the vast majority have a life expectancy of more than 60 years.

Most patients die suddenly and this is often the first manifestation of the disease (in adolescents and young adults). About 40% of deaths occur after exercise, 60% at rest.

With ischemic cardiomyopathy, there is a dependence of patient survival on the volume of viable myocardium, which can be increased by performing coronary artery bypass grafting. If ischemic cardiomyopathy is considered, the cause of death is thromboembolism and severe heart failure. Coronary artery bypass grafting has become a common cardiac surgery. Thanks to the restoration of myocardial vascularization, angina attacks are eliminated in patients, but the contractile function of the myocardium does not improve, and, accordingly, the main cause of death is not eliminated.

In this regard, patients undergo a combined operation - myocardial revascularization and reconstruction of the left ventricle (intraventricular plasty with a patch). This is what allows you to improve myocardial function, reduce the size and shape of the left ventricle. Successful surgical treatment enables reverse myocardial remodeling.

The prognosis of congenital cardiomegaly is uncertain, since infants die in 30% of cases, and survivors have serious cardiac dysfunction, but compatible with life. In children with dilated cardiomyopathy who fell ill before 2 years of age, the survival rate is 89.6% in the first 5 years, the rest have a complete recovery.

In children who fell ill at school age, the survival rate is much lower - no more than 22% survive in the first 5 years. According to statistics, the average life expectancy, if we take the time from the onset of the disease, is 19.5 months. The cause of death in children is most often heart failure, which is not amenable to medical treatment, in second place is thromboembolism.

List of sources

• Sabirov L. F. et al. Dilated cardiomyopathy // Bulletin of modern clinical medicine. 2012. V. 5, No. 3. S. 56–63.
• Moiseev V.S., Kiyakbaev G.K., Lazarev P.V. Cardiomyopathy and myocarditis - GEOTAR-Media, 2016.- 532 p.
• Amosova E.N. Cardiomyopathy. - K .: Book Plus, 1999. - 421 p.
• Amosova E.N. The course of dilated cardiomyopathy // Klin, med. -1991.- N.3.- P.48.
• Frolova E. B., Yaushev M. F. Modern concept of chronic heart failure // Bulletin of modern clinical medicine. 2013. V. 6, No. 2. S. 87–93.

Cardiomyopathy is a pathology of the heart muscle, which is based on various causes.

The disease can develop due to genetic disorders, hormonal changes, toxic effects of drugs, alcohol, and other pathological conditions.

Cardiomyopathy as a separate nosology has a common code according to ICD 10, indicated by column I42.

Types and manifestations

The classification of myocardial changes is based on the identification of pathogenetic mechanisms for the formation of cardiac disorders.

There are family cardiomyopathies, the cause of which lies in hereditary factors. Hypertrophy with subsequent wear of the heart muscle in athletes is not uncommon.

Contrary to the frequent asymptomatic course of the disease, cardiopathy can cause sudden death among the full "health" of a person.

Usually, pathology is diagnosed when patients present with characteristic cardiac complaints of palpitations, retrosternal pain, general deterioration, weakness, dizziness, and fainting.

What changes are taking place

There are several response processes from the myocardium when exposed to a damaging etiological factor:

• the heart muscle can hypertrophy;
• the cavities of the ventricles and atria are overstretched and expanded;
• directly due to inflammation, myocardial restructuring occurs.

Morphologically altered heart tissues are not able to adequately provide blood circulation. Heart failure and/or arrhythmia comes to the fore.

Diagnosis and treatment

Cardiomyopathy is diagnosed on the basis of the patient's history with additional studies. One of the screening methods are ECG (if necessary with daily monitoring) and echocardiography (using ultrasound). To determine the cause of the disease, study laboratory parameters of blood and urine.

Therapy of cardiopathy consists in the symptomatic treatment of the main manifestations. For this purpose, tablet forms of antiarrhythmics, diuretics, and cardiac glycosides are prescribed. To improve the nutrition of the myocardium, vitamins, antioxidants, and metabolic agents are prescribed.

To facilitate the work of the heart, drugs that reduce vascular resistance (calcium antagonists and beta-blockers) are used.

If necessary, surgical intervention is performed to install the pacemaker.

Code in the list of diseases

Among diseases of the circulatory system (Article IX, I00-I99), the diagnosis "Cardiomyopathy" of the ICD is singled out as a separate subsection of other heart diseases along with large nosological groups.

The division into cardiopathy, depending on the manifestations and etiology, is reflected in the international list of diseases through a dot after the main code.

So cardiomyopathy, which developed on the basis of long-term use of drugs, is coded according to ICD 10 as I42.7.

Myocardial pathology is often detected as part of the symptom complex of various diseases.

If cardiac disorders become part of a separate nosology, then in ICD 10 cardiomyopathy can be encrypted under the heading I43.

ICD-10 was introduced into healthcare practice throughout the Russian Federation in 1999 by order of the Russian Ministry of Health dated May 27, 1997. №170

The publication of a new revision (ICD-11) is planned by WHO in 2017 2018.

With amendments and additions by WHO.

Processing and translation of changes © mkb-10.com

Cardiomyopathy: what does the diagnosis mean, how is the ICD 10 code deciphered

Cardiomyopathy is a pathology of the heart muscle, which is based on various causes.

The disease can develop due to genetic disorders, hormonal changes, toxic effects of drugs, alcohol, and other pathological conditions.

Cardiomyopathy as a separate nosology has a common code according to ICD 10, indicated by column I42.

Types and manifestations

The classification of myocardial changes is based on the identification of pathogenetic mechanisms for the formation of cardiac disorders.

There are family cardiomyopathies, the cause of which lies in hereditary factors. Hypertrophy with subsequent wear of the heart muscle in athletes is not uncommon.

Contrary to the frequent asymptomatic course of the disease, cardiopathy can cause sudden death among the full "health" of a person.

Usually, pathology is diagnosed when patients present with characteristic cardiac complaints of palpitations, retrosternal pain, general deterioration, weakness, dizziness, and fainting.

What changes are taking place

There are several response processes from the myocardium when exposed to a damaging etiological factor:

• the heart muscle can hypertrophy;
• the cavities of the ventricles and atria are overstretched and expanded;
• directly due to inflammation, myocardial restructuring occurs.

Morphologically altered heart tissues are not able to adequately provide blood circulation. Heart failure and/or arrhythmia comes to the fore.

Diagnosis and treatment

Cardiomyopathy is diagnosed on the basis of the patient's history with additional studies. One of the screening methods are ECG (if necessary with daily monitoring) and echocardiography (using ultrasound). To determine the cause of the disease, study laboratory parameters of blood and urine.

Therapy of cardiopathy consists in the symptomatic treatment of the main manifestations. For this purpose, tablet forms of antiarrhythmics, diuretics, and cardiac glycosides are prescribed. To improve the nutrition of the myocardium, vitamins, antioxidants, and metabolic agents are prescribed.

To facilitate the work of the heart, drugs that reduce vascular resistance (calcium antagonists and beta-blockers) are used.

If necessary, surgical intervention is performed to install the pacemaker.

Code in the list of diseases

Among diseases of the circulatory system (Article IX, I00-I99), the diagnosis "Cardiomyopathy" of the ICD is singled out as a separate subsection of other heart diseases along with large nosological groups.

The division into cardiopathy, depending on the manifestations and etiology, is reflected in the international list of diseases through a dot after the main code.

So cardiomyopathy, which developed on the basis of long-term use of drugs, is coded according to ICD 10 as I42.7.

Myocardial pathology is often detected as part of the symptom complex of various diseases.

If cardiac disorders become part of a separate nosology, then in ICD 10 cardiomyopathy can be encrypted under the heading I43.

How is functional cardiomyopathy classified according to ICD 10?

Cardiomyopathy (ICD-10 code: I42) is one of the types of myocardial diseases. In the medical field, this disease is called dysmetabolic cardiomyopathy. The main provocateur of a group of diseases is a disturbed metabolism, localized precisely in the region of the heart.

During the onset of the disease, not only the heart muscle, but also other vital organs are subjected to “stress”. A negative process occurs in the area of ​​\u200b\u200bparts of the heart, and especially in the place where a large number of vessels are located. Such disorders cause myocardial disease, resulting in endotoxicosis.

Most often, the disease affects young people whose activities are related to sports. The development of the disease occurs during prolonged physical stress, the functional ability of the body weakens with a lack of vitamins in the body or with hormonal failure.

Cardiomyopathy is present in the international classification of diseases. The list includes various types associated with disorders in important body systems. The 10th revision of the International Classification of Diseases (ICD-10) includes such groups as:

• alcoholic cardiomyopathy;
• restrictive form;
• unspecified cardiopathy.

Cardiomyopathy is a group of inflammatory processes that are localized in the area of ​​muscle tissue. For a long time, doctors could not determine the exact causes of the onset of the disease. That is why experts decided to identify a number of reasons that, under the influence of certain conditions and circumstances, cause a violation of the myocardium.

Each of the types (dilated, hypertrophic or restrictive) cardiomyopathy has characteristic features. But the treatment is the same for the entire group of diseases and is aimed at eliminating symptoms and chronic heart failure.

The development of the disease occurs under the influence of various factors. Myocardial damage in cardiomyopathy can act as a primary or secondary process as a result of exposure to systemic ailments. All this is accompanied by the development of insufficiency in the heart and in rare cases leads to death.

There are primary and secondary causes of the disease.

The cause of primary cardiomyopathy is a congenital pathology of the heart, which can develop as a result of laying myocardial tissue while the child is still in the womb. There may be enough reasons for this: starting from the bad habits of a woman and ending with the appearance of depression and stress. Improper nutrition before and during pregnancy can have a negative impact.

The primary causes also include a mixed and acquired form.

As for the secondary causes, this includes accumulation cardiopathy, toxic, endocrine and alimentary forms of the disease.

Symptoms of the disorder can appear at any age. Basically, they are not pronounced, so some people do not experience ailments until a certain point.

According to statistics, patients with cardiomyopathy have a normal life expectancy and often live to a ripe old age. But when exposed to negative factors, complications can appear.

The symptoms of the disease are quite common, and it is very easy to confuse cardiomyopathy with other ailments. In the initial stages, a person suffers from shortness of breath on exertion, chest pain, and dizziness. Weakness is another common symptom. Such symptoms may occur due to dysfunction - a violation of the ability of the heart to contract.

Even if these symptoms appear, you should consult a specialist, as the consequences can be serious. If chest pain has acquired a aching character and is accompanied by severe shortness of breath, this is the first serious manifestation of a group of diseases.

With a lack of air during exercise and sleep disturbances, you need to think about the presence of any disease and consult a doctor.

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ICD 10 cardiomyopathy

CARDIOMYOPATHY.

Myocardial dystrophy with menopause.

Myocardial dystrophy in thyrotoxicosis.

G62.1 Alcoholic myocardial dystrophy.

The causes are diseases and conditions that lead to depletion, mutation and a decrease in the efficiency of the muscle cells of the heart.

Hypovitaminosis and beriberi (insufficient intake or lack of vitamins in the body).

Myasthenia gravis, myopathy (neuromuscular disorders).

Toxic poisoning (carbon monoxide, barbiturates, alcoholism, drug addiction).

Thyrotoxicosis (thyroid disease).

Endocrine disorders (violation of protein, fat and carbohydrate metabolism).

Violations of the water and electrolyte balance (dehydration).

Violation of the hormonal background (menopause period).

Pathogenesis and pathological anatomy.

1. Violation of the central regulation leads to an increase in myocardial oxygen demand.
2. Reduced ATP production and oxygen use.
3. Activation of peroxidation, accumulation of free radicals leads to further damage to the myocardium.

Shortness of breath on exertion.

Decreased performance and exercise tolerance.

Heart rhythm disturbances (arrhythmias).

There may be swelling in the legs.

Expanding the boundaries of the heart.

Heart sounds are muffled, systolic murmur at 1 point.

The most common variants of myocardial dystrophy.

Long-term use of alcohol (chronic alcoholism) leads to disruption of cellular structures and metabolic processes in the myocardium.

It develops in women after 45 - 50 years (during menopause or after it).

There is no specific diagnosis of myocardial dystrophy.

The diagnosis is made on the basis of:

ECG signs are an increase in heart rate (tachycardia), arrhythmia and T wave smoothness;

X-ray of the heart: increase in size;

Cardiomyopathy (CMP) is a disease of the myocardium, accompanied by its dysfunction.

In 2006, the American Heart Association (AHA - American Heart Association) proposed a new definition of CMP.

Cardiomyopathy is a heterogeneous group of diseases of various etiologies (often genetically determined), accompanied by mechanical and / or electrical dysfunction of the myocardium and disproportionate hypertrophy or dilation.

Arrhythmogenic dysplasia of the right ventricle;

ANA also proposed a new classification of the ILC:

Primary cardiomyopathy is a disease in which there is an isolated lesion of the myocardium.

Secondary cardiomyopathy is a myocardial lesion that develops with a systemic (multi-organ) disease.

HCM is a hereditary disease characterized by asymmetric hypertrophy of the left ventricular myocardium.

I 42.1. Obstructive hypertrophic cardiomyopathy.

I 42.2. Other hypertrophic cardiomyopathy.

HCM is an inherited disease that is transmitted as an autosomal dominant trait. Currently, about 200 mutations responsible for the development of the disease have been identified.

chest pain (36-40%);

dizziness, which was regarded as a presyncope state (14–29%);

syncope (36–64%);

Inspection. On examination, there are no characteristic clinical signs.

Palpation. A high, diffuse apex beat is determined, which is often shifted to the left.

Auscultation: systolic murmur, which is detected at the apex and in the fourth intercostal space to the left of the sternum.

LABORATORY STUDIES No changes.

DNA analysis of mutant genes is the most accurate method for verifying the diagnosis of HCM.

From instrumental studies perform:

Electrocardiography (overload and / or hypertrophy of the LV myocardium, rhythm and conduction disturbances),

x-ray examination of the chest: signs of enlargement of the left ventricle and left atrium,

Holter ECG monitoring,

EchoCG is the "gold" standard in the diagnosis of HCM;

Magnetic resonance imaging is indicated for all patients before surgery.

coronary angiography. It is performed with HCM and persistent retrosternal pain (frequent attacks of angina pectoris).

INDICATIONS FOR CONSULTATION OF OTHER SPECIALISTS

To exclude genetic diseases and syndromes, patients should be referred to a genetic counseling specialist.

HCM must be differentiated from diseases accompanied by LV myocardial hypertrophy.

Dilated cardiomyopathy (DCM) is a primary myocardial lesion that develops as a result of various factors (genetic predisposition, chronic viral myocarditis, immune system disorders) and is characterized by a pronounced expansion of the heart chambers.

I 42.0. dilated cardiomyopathy.

The incidence of DCMP is 5–7.5 cases per year in the population. In men, the disease occurs 2-3 times more often, especially at the age of 30-50 years.

Cardiomyopathy (I42)

Excludes: cardiomyopathy complicating. pregnancy (O99.4). postpartum period (O90.3) ischemic cardiomyopathy (I25.5)

I42.0 Dilated cardiomyopathy

I42.1 Obstructive hypertrophic cardiomyopathy

Hypertrophic subaortic stenosis

I42.2 Other hypertrophic cardiomyopathy

Non-obstructive hypertrophic cardiomyopathy

I42.3 Endomyocardial (eosinophilic) disease

Endomyocardial (tropical) fibrosis Loeffler's endocarditis

I42.4 Endocardial fibroelastosis

I42.5 Restrictive cardiomyopathy other

I42.6 Alcoholic cardiomyopathy

If it is necessary to identify the cause, use an additional external cause code (class XX).

I42.8 Other cardiomyopathies

I42.9 Cardiomyopathy, unspecified

Cardiomyopathy (primary) (secondary) NOS

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Cardiomyopathy: generalized heart failure

Cardiomyopathy (ICD - 10 - disease code i42) is a pathology of the cardiovascular system, represented by a group of diseases with a common symptom in the form of damage to the heart muscle, manifested by a clinic of heart failure, arrhythmias and cardiomegaly (enlargement of the heart chambers: atria and ventricles).

cardiomyopathy

The causes of cardiomyopathy in its idiopathic variant are unknown, there is no clear relationship with the presence of inflammatory or neoplastic processes, as well as the accompanying hypertension and coronary heart disease.

Secondary cardiomyopathies have a clear relationship with a provoking factor.

The symptoms of the disease are varied and depend on the type and severity of the disorders.

Types of cardiomyopathy

Cardiomyopathy is divided into three main types depending on the course of pathological processes in cardiomyocytes with corresponding changes in the structures of the heart chambers.

• Hypertrophic
• Dilated
• Obstructive cardiomyopathy.

Hypertrophic cardiomyopathy

It is a thickening of the walls of the heart muscle, due to which the overall size of the heart can increase.

It happens diffuse (symmetrical), when the myocardium of the left ventricle is evenly and symmetrically thickened in all departments, including the department of the interventricular septum. The right ventricle is rarely involved in the process. This variant is called non-obstructive hypertrophic cardiomyopathy, since the thickened myocardium does not affect the ejection of blood from the left ventricle and intracardiac hemodynamics.

There is also an asymmetric version. It happens with obstruction and without obstruction.

Obstructive cardiomyopathy develops due to thickening of the upper wall of the interventricular septum. As a result, during heart contractions, this enlarged part blocks the lumen for the exit of blood from the cavity of the left ventricle. Gradually disturbed intracardiac hemodynamics (blood circulation in the cavities). This form is also called idiopathic hypertrophic subaortic stenosis.

Apical hypertrophic cardiomyopathy is observed with thickening of the lower part of the myocardium of the left ventricle in the region of the apex of the heart.

Unfortunately, the prognosis is rather pessimistic when such a diagnosis as hypertrophic cardiomyopathy is revealed. The average life expectancy with this form of the disease will be about 15-17 years, which is slightly higher than with the dilated variant (does not exceed an average of 5-7 years).

However, the earlier the disease is detected, the higher the percentage of survival and the effectiveness of the treatment.

To exclude pathology in the event of suspicious symptoms, it is recommended to immediately contact a specialist for examination and timely diagnosis.

Dilated cardiomyopathy

Its main manifestation is the expansion of intracardiac cavities with thinning of the walls and a violation of their contractile function.

Another name for this variant is congestive cardiomyopathy: due to the development of myocardial weakness, congestion occurs in the body with the formation of persistent peripheral edema and edema of the internal organs, up to the development of anasarca.

The causes of dilated cardiomyopathy are most often associated with hereditary predisposition and immune defects in the regulation of the body's defenses against infectious factors. As a result of exposure to an unknown cause, some of the myocardial cells die, followed by their replacement with fibrous tissue and the gradual “spreading” of the walls of the heart chambers in diameter. The myocardium becomes like a "rag".

Dilated cardiomyopathy in children is the most common compared to other forms of the disease.

Restrictive cardiomyopathy

This form combines manifestations of fibrosis (compaction) of the myocardium and endocarditis (inflammation of valve structures). The walls of the heart become inactive and thick, the ability to contract muscle fibers decreases. A common cause of such changes is the impregnation of the myocardium with amyloid (a specific protein in amyloidosis). As a result, even in diastole, the heart does not relax (does not stretch from blood flow), which is also manifested by cardiovascular disorders.

There is another division of the entire pathology into forms of cardiomyopathy, the classification of which reflects the main causative factor of the disease. The disease is divided into idiopathic (primary) and secondary variants.

Idiopathic cardiomyopathy

This is most often congenital cardiomyopathy, the causes of which are not known.

Cardiomyopathy in newborns can develop as a result of intrauterine infections of a viral or bacterial nature. Also, such changes in the heart muscle can appear as a result of exposure to certain drugs or chemicals, intoxication, and a lack of nutrients. The influence of genetic factors and metabolic disorders cannot be excluded.

Cardiomyopathy in children can occur in the form of any form of pathology, causing severe suffering and health problems: even death.

To help children suffering from cardiomyopathy and other chronic heart diseases, the National Organization on a non-profit basis CCF was created. The main directions in her work are targeted assistance to sick babies, as well as an active search and promotion of new methods for diagnosing and treating cardiomyopathy in children.

Secondary cardiomyopathy

This is cardiomyopathy, the causes of which are known and can be established in a particular patient. Among them, the main ones are the effects of alcohol, toxins, ischemia, metabolic products.

Secondary cardiomyopathy, death from which is recorded in 15% of cases among other forms of cardiomyopathy, is divided into alcoholic, dishormonal, ischemic and toxic.

Alcoholic cardiomyopathy

It develops as a result of prolonged exposure to alcohol (ethanol) on cardiomyocytes. It is more common in men with alcoholism. In the myocardium, dystrophic processes develop with a gradual decrease in myocardial contractility and weakness of the heart muscle. Often accompanied by hepatitis, cirrhosis of the liver and pancreatitis, which also negatively affect the overall metabolism.

Dishormonal cardiomyopathy

It develops with metabolic disorders in the body. The causes may be hormonal diseases such as hypo- or hyperthyroidism (thyroid disease with low or high production of thyroid hormones), menopause in women, as well as metabolic disorders of electrolytes, vitamins and trace elements.

Cardiomyopathy occurs during pregnancy (myocarditis is diagnosed), when dystrophic changes are observed in the myocardium due to an imbalance of hormones. One form of dilated cardiomyopathy. After childbirth, in half of the cases it stops on its own, the other half progresses to serious congestion and the need for a heart transplant.

Ischemic cardiomyopathy

Develops as a final coronary heart disease. It is one of the varieties of dilated cardiomyopathy. When collecting an anamnesis, attention is paid to the course of symptoms typical of coronary artery disease, as well as signs of myocardial ischemia during additional examination methods.

Toxic cardiomyopathy

It can be caused by exposure to toxins, as well as physical factors (for example, ionizing radiation) on myocardial cells. Developing inflammation with the transition to necrosis and fibrosis leads to the formation of secondary dilated cardiomyopathy.

Tonsillogenic cardiomyopathy

Diseases of the tonsils or adenoids can lead to metabolic disorders in the heart muscle. Chronic tonsillitis can cause the development of cardiomyopathy with degenerative processes in the myocardial stroma. Often develops in childhood.

Degrees of compensation for cardiomyopathy

The classification presented below evaluates the general condition of patients in accordance with the severity of the disease. There are three stages:

1 - stage of compensation, clinic with minimal symptoms, hemodynamics at the proper level.

2 - stage of subcompensation, more pronounced symptoms of the disease appear with minor hemodynamic disturbances. In the heart muscle, dystrophic processes are moderately expressed.

3 - stage of decompensation, when ordinary physical activity causes serious hemodynamic changes, and systolic dysfunction of the myocardium is expressed significantly or sharply.

Who treats this disease?

Treatment of cardiomyopathy is aimed at stopping the further progression of the pathology, correcting symptoms and preventing the development of complications.

When establishing the diagnosis of "cardiomyopathy", recommendations for treatment are given by specialist doctors of the following specialties:

• Cardiologist, if surgery is necessary, a cardiac surgeon or transplantologist
• Therapist
• Family doctor

Why is cardiomyopathy dangerous?

Death in this pathology can occur suddenly. This is explained by the fact that acute cardiomyopathy can be complicated by severe arrhythmias and cardiac conduction disorders that are incompatible with life. These include ventricular fibrillation and the development of a complete atrioventricular block.

Acute myocardial infarction often develops with hypertrophic cardiomyopathy. With its extensive distribution, acute heart failure develops.

Cardiomyopathy itself is not a major cause of death. Deadly complications develop against its background.

Complications of cardiomyopathies

The main and dangerous complication of cardiomyopathy is the development of rhythm disturbances. On their basis, more severe pathologies are added. For example, due to violations of intracardiac hemodynamics, diseases such as infective endocarditis, thrombosis and embolism in various vessels often develop with a clinic of cerebral stroke or heart attack of the heart, kidney, spleen and other internal organs.

In the final, chronic heart failure often develops.

Difficulties of modern medicine

Cardiomyopathy of the heart has many causative factors that directly affect the course and outcome of the disease. A large number of serious complications makes the prognosis of cardiomyopathy generally unfavorable.

The patient always has an increased risk of sudden death from thromboembolism or fatal arrhythmias.

In addition, the signs of heart failure are gradually becoming more pronounced every year.

Of course, medicine has come a long way. And where the five-year survival rate was barely 30%, now with early detection and adequately prescribed treatment, it is possible to extend the patient's life for a longer period.

The branch of transplantology is developing widely, giving hope to such patients. There are cases after a heart transplant with life extension for more than one decade.

With the help of surgery, it is possible not only to replace the heart "pump". Positive results have methods for eliminating obstruction of the outflow tract from the left ventricle in hypertrophic cardiomyopathy.

Cardiac cardiomyopathy is a direct contraindication to pregnancy in women, since maternal mortality in this pathology is very high.

It is not yet possible to prevent the development of cardiomyopathy due to the lack of knowledge about the causes of its occurrence.

Preventive measures are of a general nature and are aimed at stimulating the immune defense, preventing infection with infectious agents and maintaining a healthy lifestyle.

Symptoms of cardiomyopathy

Symptoms of cardiomyopathy are:

• the appearance of shortness of breath (especially during physical exertion);
• increased fatigue;
• swelling of the legs;
• causeless cough;
• pallor of the skin;
• blue fingers on the hands;
• the appearance of pain in the chest;
• frequent fainting;
• increased sweating;
• a significant decrease in performance;
• increased heart rate;
• dizziness;
• sleep disorder and others.

Treatment of cardiomyopathy is long and rather complicated. It depends on the form of the disease, the age of the person and the severity of his condition. Cardiomyopathy in children can be congenital. It develops as a result of a violation of embryogenesis. The prognosis for this disease is not always good. Over time, there is an exacerbation of the manifestation of all symptoms, which leads to the development of pathologies that are incompatible with life.

Dilated cardiomyopathy - symptoms

Dilated cardiomyopathy is most often observed in people of working age (20-40 years). But it is also possible that this disease is diagnosed in children or the elderly. Symptoms of the development of cardiomyopathy of this form are:

• the appearance of shortness of breath, which is most often manifested during physical exertion;
• increased heart rate;
• the appearance of edema;
• the development of orthopnea - difficulty breathing at rest, which manifests itself in a horizontal position;
• complete intolerance to physical activity;
• asthma attacks that appear at night. This condition resembles that observed in bronchial asthma;
• fast fatiguability;
• on palpation, there is a slight increase in the liver;
• swelling of the veins in the neck;
• blue fingertips;
• increased urination in the evening and at night;
• decrease in working capacity;
• muscle weakness;
• feeling of heaviness in the lower extremities;
• increase in the size of the abdominal cavity;
• feeling of discomfort in the right hypochondrium.

Approximately 3-12% of patients with this disease proceed without pronounced symptoms. Despite this, pathological processes in the cardiac and vascular system are aggravated every day. This is especially dangerous when, when the first symptoms of cardiomyopathy appear, it cannot be diagnosed. But with the progression of the disease, its signs are determined in 90-95% of cases.

Dilated cardiomyopathy can progress slowly. In this case, there is a slight increase in the heart, which is accompanied by mild shortness of breath, exercise intolerance, swelling of the extremities. With rapidly progressive cardiomyopathy, acute heart failure, arrhythmia, suffocation and other symptoms are detected. In this case, most often the disease ends with the death of a person in about 2-4 years.

Hypertrophic cardiomyopathy in humans - symptoms

Signs of this type of cardiomyopathy in most cases are almost invisible, but as the disease progresses, they become more pronounced. First of all, a person has shortness of breath. It is manifested by some violation of respiratory activity, which can even turn into attacks of suffocation. At the initial stage of the disease, this symptom is observed with significant physical exertion or during stress. But as hypertrophic cardiomyopathy progresses, shortness of breath develops even at rest. Due to the accumulation of blood in the vessels of the lungs, their edema is observed. In this case, wet rales and cough appear. At the same time, sputum with foam is expectorated.

Also, with hypertrophic cardiomyopathy, the heart rate increases significantly. Usually, at rest, a person does not feel the beating of his heart, but with the development of this disease, it is felt even in the upper abdomen and at neck level. Due to the deterioration of the heart muscle, other tissues are not supplied with enough blood. This is accompanied by pallor of the skin. In parallel with this, the limbs and nose are always cold, fingers may turn blue. It also causes significant muscle weakness and fatigue.

Another symptom of this disease is the formation of edema in the lower extremities, enlargement of the liver, spleen. Also, at the initial stage of hypertrophic cardiomyopathy, there are pains in the sternum due to oxygen starvation of the heart. At the first stage, a person feels slight discomfort after exercise. Over time, pain appears even at rest.

As hypertrophic cardiomyopathy progresses, oxygen starvation of the brain develops due to circulatory disorders. In this case, the sick person constantly feels that he is dizzy. Also, with hypertrophic cardiomyopathy, there are frequent cases of sudden fainting. This happens due to a sharp drop in blood pressure due to the fact that the heart stops pumping blood.

Symptoms of Restrictive Cardiomyopathy

With restrictive cardiomyopathy, there are signs of severe heart failure, which is accompanied by the following symptoms:

• severe shortness of breath even at rest or with little physical exertion;
• swelling in the lower extremities;
• accumulation of fluid in the peritoneum;
• an increase in the size of the liver, which is determined by palpation;
• fast fatiguability;
• muscle weakness;
• swelling of the veins in the neck;
• a significant decrease in appetite, and in some cases its complete absence;
• the appearance of pain in the abdominal cavity. In this case, the patient cannot determine their localization;
• causeless weight loss;
• the appearance of nausea, sometimes vomiting;
• the development of a dry cough, which may be accompanied by the release of a small amount of sputum in the form of mucus;
• there is a slight itching of the skin, formations appear that are characteristic of urticaria;
• increase in body temperature;
• excessive sweating especially at night;
• dizziness, which may be accompanied by headaches.

With restrictive cardiomyopathy, general symptoms first appear, which do not indicate a violation of the normal functioning of the heart. As the disease progresses, other signs are connected that already clarify the picture of what is happening.

Signs of alcoholic cardiomyopathy

Alcoholic cardiomyopathy develops against a background of serious dependence, when a person takes a certain dose of ethanol-containing drinks daily for several years. In this case, there is a violation of respiratory activity. The patient suffers from constant shortness of breath or attacks of suffocation, especially after some physical activity. In parallel with this symptom, swelling of the lower extremities, tachycardia appear.

As the disease progresses, other more serious symptoms develop that indicate pathological changes in all systems:

• instability of the emotional state;
• increased excitability;
• in some cases, excessive activity and talkativeness;
• increased fussiness;
• significant irritability;
• sleep disturbance, the appearance of insomnia;
• inappropriate behavior;
• significant pain in the chest, where the heart is localized;
• hand tremors;
• feet and hands are always in a cold state;
• redness and even some cyanosis of the skin;
• increased sweating;
• jumps in blood pressure;
• severe headaches, migraines;
• dry cough, which leads to pain in the sternum.

Dishormonal cardiomyopathy - symptoms

Dishormonal cardiomyopathy is accompanied by the appearance of unpleasant pulling pains in the chest in the region of the heart. They suddenly become sharp and take on a stabbing character. The pain is very intense, often radiating to the left shoulder blade, arm and lower jaw. This condition can be observed from several hours to 2-3 days. Pain can only be relieved with painkillers. Its appearance does not depend on physical activity and can overtake even during a period of complete rest.

Also, a sick person feels that he is getting hot. Unpleasant sensations are localized in the upper body in the face and chest. Because of this, the person sweats a lot. This state is replaced by chills, the limbs become cold. Most often, such a person has a drop in blood pressure, tachycardia, dizziness, tinnitus.

Treatment of cardiomyopathy

Cardiomyopathy is a dangerous disease that very often leads to death in patients. The causes of this disease have not been fully identified, however, it has been established that disorders in the development of the heart are often transmitted genetically. Therefore, prevention of cardiomyopathy is not always effective. It can be carried out only when planning a pregnancy in the case of existing similar diseases in the family. As a preventive measure, genetic counseling is carried out.

There are several types of cardiomyopathy, depending on which pathologies arose in which parts of the heart. There are dilated, hypertrophic and restrictive cardiomyopathy. According to the results of the diagnosis, it is imperative to determine what type of cardiomyopathy has occurred in the patient in order to prescribe the correct treatment regimen for the disease.

Treatment of dilated cardiomyopathy

First of all, in the treatment of dilated cardiomyopathy, it is necessary to eliminate heart failure, which is characteristic of this type of disease. This will help to avoid further complications of the disease. In the treatment of heart failure, beta-blockers are used, which prevent the absorption of stress hormones, such as adrenaline, for example.

At the same time, angioprosthetic enzyme inhibitors should be prescribed, which will not allow an increase in blood pressure. The most commonly prescribed drug is Renitec or Cardopril, as they are highly effective and have fewer side effects.

In case of individual intolerance to ACE inhibitors, blockers of receptors that react to this enzyme are prescribed. This is a less effective, but safer way to maintain normal blood pressure. A cardiologist who oversees the course of the disease should choose one or another drug to lower pressure. He will make a choice based on tests and individual patient indicators. Self-selection of drugs is fraught with the development of serious complications.

Antioxidants have a positive effect on the myocardium. Therefore, they are also often used in the complex therapy of dilated cardiomyopathy. In the presence of swelling during the disease, it is very important to control the volume of urination of the patient in accordance with his weight. If during the treatment it is established that the fluid is retained in the body, it is necessary to start taking diuretics. Within a week, with the right dosage, the puffiness should subside.

With small indicators of puffiness, it is possible to be treated not only with medications, but also with folk remedies. Of course, only after consulting a doctor on this issue. Among folk diuretics, elderberry, oregano, clover and much more are distinguished. Alternative methods are no less effective than medicinal ones and have fewer contraindications. Homeopathic methods of treatment are also practiced, however, studies of this method of treatment have not shown positive results.

In the treatment of dilated cardiomyopathy, a study of the electrolyte composition of the blood should be carried out regularly, which will indicate its effectiveness.

Despite the correctly selected medical method of treatment, the prognosis of this disease is always negative. Most patients die in the first 5 years after the onset of the main symptoms. In addition, women suffering from dilated cardiomyopathy are contraindicated in pregnancy for several reasons. First, the disease is genetically transmitted and can occur in a child. Secondly, during pregnancy, the disease often begins to progress dramatically. Thirdly, childbirth with such a diagnosis often ends in death for the mother, since the heart cannot withstand excessive stress.

That is why a heart transplant is necessary for effective treatment. However, today the queue for a donor heart is so long that patients often do not have time to live before their operation and only a few manage to get a new heart.

The current treatment for cardiomyopathy is stem cell therapy. These cells are versatile and can be used as building material for any organ and body part. The donor of stem cells is the patient himself after a thorough examination. These cells are extracted from bone marrow or adipose tissue and left in a special laboratory for further reproduction.

For effective treatment, a large number of stem cells are required. Therefore, their reproduction takes some time. In total, at least 100 million cells are needed to start therapy. However, when the required number of cells appears, they still need to be prepared. To do this, with the help of special chemical compounds, the cells are converted into cardioblasts - the building material of the heart muscle.

Part of the finished material is frozen and stored for some time. This is necessary in case of recurrence of the disease and repeated therapy. The cells are injected intravenously, after which they themselves find the affected area and restore it.

Treatment of hypertrophic cardiomyopathy

The entire treatment regimen for hypertrophic cardiomyopathy is aimed at improving the contractility of the left heart ventricle. For this purpose, most often a drug such as Verapamil or Diltiazem is prescribed. They reduce the number of heartbeats by slowing down the pulse and even out the rhythm of the heartbeat. Often, the drug Disopramide is added to the classic drug treatment regimen, which enhances the effect of the main drug.

In more serious forms of hypertrophic cardiomyopathy, a pacemaker is implanted, which produces electrical impulses and normalizes the heart rate. This is the most effective way to treat hypertrophic cardiomyopathy, however, it is prescribed only if medical treatment is not effective.

Treatment of restrictive cardiomyopathy

Restrictive cardiomyopathy is the most difficult in terms of treatment. Due to the fact that the disease manifests itself too late. Compared to other stages, treatment is most often no longer effective. By the time the disease is finally diagnosed, the patient becomes disabled and, as a rule, does not live for more than 5 years.

Therefore, doctors do not know how to treat cardiomyopathy in this case, since all treatment can only be aimed at relieving symptoms and improving the patient's quality of life and slightly prolonging its period.

Reliable methods for completely stopping the pathological process have not yet been found. The fact is that even an implant placed may not save the situation, since relapses of the disease often occur on the implanted heart. When queuing for heart implantation, doctors take into account the specifics of this type of disease, and the advantage remains in patients with other less complex types of diseases. Therefore, surgery for this type of cardiomyopathy is a very rare case in medical practice.

Thus, the effectiveness and method of treatment depends on the type of disease, and the prognosis of treatment can also be different. In general, cardiomyopathy is a dangerous disease that can be fatal even with proper treatment.

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Dilated cardiomyopathy(DCMP) is a primary lesion of the heart, characterized by the expansion of its cavities and impaired contractile function.

Statistical data

The incidence in the world is 3-10 cases per 100,000 people per year. Men get sick more often than women (2:1).

Code according to the international classification of diseases ICD-10:

• I42. 0 - Dilated cardiomyopathy

Dilated Cardiomyopathy: Causes

Etiology

The occurrence of DCMP is associated with the interaction of several factors: genetic disorders, exogenous influences, autoimmune mechanisms.

exogenous factors. A connection between the transferred infectious myocarditis and the development of DCMP was revealed. It has been established that DCMP can develop after myocarditis (in 15% of cases) as a result of exposure to infectious agents (enteroviruses, Borrelia, HCV, HIV, etc.). Using the technique of molecular hybridization, enteroviral RNA was detected in nuclear DNA in patients with myocarditis and DCMP. After an infection caused by Coxsackie viruses, heart failure may develop (even after several years). Convincing evidence has been obtained that the toxic effect of alcohol on the myocardium can lead to the onset of DCM. In experimental studies, exposure to ethanol or its metabolite acetaldehyde causes a decrease in the synthesis of contractile proteins, damage to mitochondria, the formation of free radicals and damage to cardiomyocytes (an increase in the content of troponin T in the blood is observed as a sign of myocardial damage). However, it should be borne in mind that severe myocardial damage of the DCM type occurs only in a proportion of persons (1/5) who abuse alcohol. . Chronic exposure to ethanol causes a decrease in protein synthesis, damage to the sarcoplasmic reticulum, and the formation of toxic fatty acid esters and free radicals. In addition, chronic alcohol consumption causes malnutrition and malabsorption leading to thiamine deficiency, hypomagnesemia, and hypophosphatemia. These disorders cause changes in the energy metabolism of cells, the mechanism of excitation - contraction and increase myocardial dysfunction.

autoimmune disorders. Under the influence of exogenous factors, heart proteins acquire antigenic properties, which stimulates the synthesis of antibodies and provokes the development of DCMP. In DCM, an increase in the content of cytokines in the blood, an increased number of activated T-lymphocytes were found. In addition, antibodies to laminin, heavy chain myosin, tropomyosin, and actin are detected.

Genetic Aspects

Familial DCM, in the development of which the genetic factor apparently plays a decisive role, is observed in 20-30% of all cases of this disease. Several types of familial forms of DCM with different genetic disorders, penetrance and clinical manifestations have been identified.

. cardiomyopathy familial dilatation: . type 1B, CMD1B, CMPD1, FDC, 600884, 9q13; . type 1C: CMD1C, CMPD3, 601493, 10q21 q23; . type 2: CMPD2, 601494, 1q32; . conduction defect type 2: CDCD2, CMPD2, 601154, 3p25 p22; . with a conduction defect, type 1: CMD1A, CDCD1, 115200 (a - cardiac actin), 1p11 q11.

. cardiomyopathy X - linked dilatation (Bart's syndrome). Clinically: DCMP, multiple myopathies, endocardial fibroelastosis, heart failure, neutropenia (arrest of differentiation at the myelocyte stage), growth retardation, pyoderma, defective mitochondria. Laboratory: in a number of patients urinary excretion of 3 - methylglutarate is detected.

Pathogenesis

Under the influence of exogenous factors, the number of fully functioning cardiomyocytes decreases, which leads to an expansion of the heart chambers and a violation of the contractile function of the myocardium. The cavities of the heart expand, which leads to the development of systolic and diastolic dysfunction of both ventricles). Chronic heart failure develops.

At the initial stages of the disease, the Frank-Starling law applies (the degree of diastolic stretching is proportional to the force of contraction of myocardial fibers). Cardiac output is also maintained by increasing heart rate and reducing peripheral resistance during exercise.

Gradually, compensatory mechanisms are violated, heart rigidity increases, systolic function worsens, and the Frank-Starling law ceases to operate. The minute and stroke volumes of the heart decrease, the end diastolic pressure in the left ventricle increases, and further expansion of the heart cavities occurs. There is relative insufficiency of the mitral and tricuspid valves due to dilatation of the ventricles and expansion of the valve rings. Compensatory myocardial hypertrophy occurs as a result of myocyte hypertrophy and an increase in the volume of connective tissue (the mass of the heart can exceed 600 g). A decrease in cardiac output and an increase in intraventricular diastolic pressure can lead to a decrease in coronary perfusion, resulting in subendocardial ischemia.

A decrease in cardiac output and a decrease in renal perfusion stimulate the sympathetic nervous and renin-angiotensin systems. Catecholamines damage the myocardium, leading to tachycardia, arrhythmias, and peripheral vasoconstriction. The renin-angiotensin system causes peripheral vasoconstriction, secondary hyperaldosteronism, leading to retention of sodium ions, fluid and the development of edema, an increase in BCC and preload.

DCM is characterized by the formation of parietal thrombi in the heart cavities: in the left atrial appendage, the right atrial appendage, the right ventricle, and the left ventricle.

Dilated Cardiomyopathy: Signs, Symptoms

Clinical manifestations

Manifestations of DCM include congestive heart failure, arrhythmias, and thromboembolism (one or all three may be present). The disease develops gradually, but in the absence of treatment (and often even against the background of treatment) steadily progresses. Complaints may be absent for a long time.

Complaints. Characteristic of chronic heart failure: shortness of breath, weakness, fatigue, palpitations, peripheral edema. When questioning patients, it is necessary to find out possible etiological points (family history, viral infection, toxic effects, other diseases, including those of the heart).

With decompensation, there are signs of stagnation in the small (shortness of breath, wheezing in the lungs, orthopnea, attacks of cardiac asthma, "gallop rhythm") and large (peripheral edema, ascites, hepatomegaly) circulation, reduced cardiac output (decrease in peripheral perfusion in the form of cyanosis and cold moist skin, low systolic blood pressure) and neuroendocrine activation (tachycardia, peripheral vasoconstriction).

One of the early manifestations of DCMP is paroxysmal atrial fibrillation (as a rule, it quickly becomes permanent).

With percussion of the heart, it is possible to reveal the expansion of the boundaries of relative cardiac dullness in both directions (cardiomegaly), with auscultation - systolic murmurs of relative insufficiency of the tricuspid and mitral valves. Arrhythmia is characterized by atrial fibrillation.

Dilated Cardiomyopathy: Diagnosis

instrumental data

ECG. Signs of hypertrophy and overload of the left ventricle (depression of the ST segment and negative T waves in I, aVL, V5, V6), left atrium. Atrial fibrillation is found in 20% of patients with DCM. Conduction disorders are possible, in particular blockade of the left leg of the His bundle (up to 80% of patients), the presence of which correlates with a high risk of sudden cardiac death (the appearance of blockade of the left leg of the His bundle is associated with the development of a fibrous process in the myocardium). Characterized by the lengthening of the interval Q-T and its dispersion. Less common is AV block.

Holter monitoring allows you to identify life-threatening arrhythmias and evaluate the daily dynamics of repolarization processes.

Echocardiography reveals the main sign of DCM — dilatation of the heart cavities with a decrease in the ejection fraction of the left ventricle. In the Doppler mode, it is possible to detect relative insufficiency of the mitral and tricuspid valves (relative insufficiency of the aortic valve may also occur), violations of the diastolic function of the left ventricle. In addition, with echocardiography, it is possible to conduct a differential diagnosis, identify the probable cause of heart failure (heart defects, postinfarction cardiosclerosis), and assess the risk of thromboembolism in the presence of parietal thrombi.

X-ray examination helps to detect an increase in the size of the heart, signs of pulmonary hypertension, hydropericardium.

Radionuclide research methods - diffuse decrease in myocardial contractility, accumulation of the radionuclide in the lungs.

MRI can detect dilatation of all parts of the heart, a decrease in the contractility of the left ventricular myocardium, venous congestion in the lungs, and structural changes in the myocardium.

Diagnostics

Diagnosis of DCM is made by ruling out other heart conditions that manifest as chronic systolic heart failure syndrome.

Differential Diagnosis

DKMP does not have any pathognomonic clinical or morphological markers, which makes it difficult to differentiate it with secondary myocardial lesions of a known nature (with coronary artery disease, arterial hypertension, myxedema, some systemic diseases, etc.). The latter, in the presence of dilatation of the chambers of the heart, are called secondary cardiomyopathies. Particularly difficult is sometimes the differential diagnosis of DCM with severe ischemic myocardial damage in relatively elderly people in the absence of a characteristic pain syndrome in the form of angina pectoris. At the same time, attention should be paid to the presence of risk factors for atherosclerosis, the presence of atherosclerotic lesions of the aorta and other vessels, but coronary angiography data may be decisive, which makes it possible to exclude stenosing lesions of the coronary arteries. Nevertheless, thanks to positron emission tomography of the myocardium, a very accurate differential diagnosis between DCM and ischemic cardiomyopathy has become possible.

Dilated Cardiomyopathy: Treatment Methods

Treatment

General tactics

Treatment of DCM consists in adequate correction of manifestations of heart failure. First of all, it is necessary to limit the amount of salt and liquid consumed. Correction of emerging rhythm disturbances is also necessary.

Drug therapy

All patients with DCMP, in the absence of contraindications, should be prescribed ACE inhibitors (captopril, enalapril, ramipril, perindopril, etc.). The drugs in this group prevent the progression of heart failure. When fluid retention occurs, ACE inhibitors are combined with diuretics, mainly furosemide.

In severe heart failure, the use of spironolactone at a dose of 25 mg / day is indicated.

In addition, digoxin can be used, especially in the presence of atrial fibrillation.

Significant difficulties in the treatment of patients with DCMP arise in the presence of persistent tachycardia and severe cardiac arrhythmias. Therapy with digoxin at doses greater than 0.25-0.375 mg/day in such patients quickly leads to the development of glycoside intoxication even at normal serum potassium concentrations. In such cases, it is advisable to use b-blockers (bisoprolol, carvedilol, metoprolol). The use of b - blockers is especially indicated for the constant form of atrial fibrillation. The favorable effect of b-blockers in DCM is evidenced by the results of a number of clinical trials, which confirmed an increase in the survival rate of patients under the influence of this group of drugs. In heart failure, the effectiveness of cardioselective drugs metoprolol and bisoprolol, as well as carvedilol, which blocks not only b-, but also a1-adrenergic receptors, has been best studied. The blockade of the latter leads to vasodilation.

Antiplatelet agents - due to the tendency to thrombosis, it is advisable to use antiplatelet agents for a long time - acetylsalicylic acid at 0.25-0.3 g / day.

Surgery

- see Chronic diastolic heart failure, Chronic systolic heart failure.

Complications

The most common complications of DCMP are: arterial and pulmonary thromboembolism (20% of patients), rhythm and conduction disturbances (30% of patients), sudden cardiac death, progressive heart failure.

Forecast

Patients with DCMP have an unfavorable prognosis in the presence of the following manifestations. Symptoms of heart failure at rest (IV functional class according to the New York classification). Pronounced dilatation of the left or right ventricle, revealed by echocardiography or x-ray examination. The spherical shape of the left ventricle according to echocardiography. Low ejection fraction of the left ventricle according to echocardiography. Low systolic blood pressure. Low cardiac index (less than 2.5 L/min/m2). High filling pressure of the left and right ventricles. Signs of pronounced neuroendocrine activation are low levels of sodium ions in the blood, increased levels of norepinephrine in the blood.

The 10-year survival rate of patients with DCMP averages 15-30%. Mortality reaches 10% per year. With an asymptomatic course of DCMP, the 5-year survival rate of patients does not exceed 80%. In patients hospitalized for chronic heart failure, the five-year survival rate is 50%. With refractory heart failure (IV functional class according to the New York classification), survival within 1 year does not exceed 50%.

Features in children. In the first 3 years of life, hereditary and idiopathic forms of DCMP most often manifest.

Pregnancy

With DCM that developed during pregnancy or the early postpartum period, re-pregnancy is contraindicated.

Synonyms

stagnant cardiomyopathy. congestive cardiomyopathy.

Reduction

DCMP - dilated cardiomyopathy.

ICD-10. I42. 0 Dilated cardiomyopathy.

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