home Diseases Hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy - description and causes of the disease, diagnosis, treatment methods and prognosis Slight hypertrophy of the intestinal tract

Hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy - description and causes of the disease, diagnosis, treatment methods and prognosis Slight hypertrophy of the intestinal tract

Hypertrophic cardiomyopathy (HCM) is a hereditary myocardial disease with massive hypertrophy of the ventricular walls (mainly the LV). Severe LV hypertrophy (as well as the common asymmetric IVS hypertrophy) is accompanied by a decrease in the size of the LV cavity, diastolic dysfunction and, often, obstruction of the LV outflow tract (subaortic stenosis).

HCM is a common cause of sudden arrhythmic death. The goal of pharmacotherapy is to improve exercise tolerance, prevent CHF, reduce obstruction, improve LV diastolic filling (calcium antagonists, beta blockers) and prevent ventricular tachyarrhythmias (amiodarone, beta blockers). Surgical interventions (myotomy, myectomy, alcohol ablation of the IVS) can reduce the degree of obstruction in the LV outflow tract and eliminate mitral regurgitation.

Keywords: left ventricular hypertrophy, family-genetic left ventricular hypertrophy, asymmetric hypertrophy of the interventricular septum.

INTRODUCTION

Hypertrophic cardiomyopathy (HCM) is a hereditary myocardial disease with massive hypertrophy of the walls of the ventricles (mainly the left) (Fig. 2.1, see inset), leading to a decrease in the size of the cavity of the left ventricle (LV), disruption of its diastolic function and, often, to obstruction LV outflow tract in patients without other diseases leading to the development of hypertrophy (arterial hypertension, COPD, heart defects, etc.).

The true incidence and prevalence of HCM are not clearly established. According to a joint consensus document between the American College of Cardiology and the European Society of Cardiology, HCM is a relatively common pathology (1:500 in the general adult population). The widespread use of echocardiography (EchoCG) has suggested that the disease is more common than previously thought and has a better prognosis than previously thought. According to some authors, echocardiographic signs of HCM are detected in 0.2% of practically healthy young people. An autosomal dominant type of inheritance has been established, and in more than 50% of cases, a similar pathology can be identified among the relatives of patients. The disease occurs almost equally in men and women, as well as in representatives of different nationalities.

HCM is a common cause of sudden death (SD) in young people (including trained athletes) and can also lead to death or disability in people of any age. According to individual centers, the annual death rate of such patients from VS is 1-2% in adults and 4-6% in children. However, even a long course of the disease can be accompanied by normal health and not change life expectancy. Due to the heterogeneity of the clinical picture, HCM may remain undiagnosed for a long time. In addition, given the relatively low frequency of this disease in the routine practice of a cardiologist, not all mechanisms of its development have been studied to date and the level of evidence for the use of certain treatment methods remains low.

When diagnosing HCM, detection of idiopathic LV hypertrophy using echocardiography and ECG, as well as identification of LV outflow tract obstruction, is of great importance.

The goal of pharmacotherapy is to improve exercise tolerance, prevent CHF, reduce obstruction by improving LV diastolic filling (calcium antagonists, beta blockers) and prevent ventricular tachyarrhythmias (beta blockers, amiodarone). Surgical intervention can reduce the degree of obstruction in the LV outflow tract and eliminate mitral regurgitation.

CLASSIFICATION

Currently, the term hypertrophic cardiomyopathy is well established. Name options such as hypertrophic obstructive cardiomyopathy, muscular subaortic stenosis, or idiopathic hypertrophic subaortic stenosis are limited in use, since some patients, especially at rest, do not have LV outflow tract obstruction.

However, the disadvantage of this classification is that it does not take into account the presence or absence of LV outflow tract obstruction. The modern classification of the disease is based on the localization of hypertrophied areas of the myocardium and the resulting hemodynamic disorders:

Idiopathic hypertrophic subaortic stenosis;

Asymmetric hypertrophy of the septum without changes in the aortic and mitral valves and without obstruction of the LV outflow tract;

Apical HCM with the hypertrophy zone limited to the apex;

Symmetrical HCM with concentric hypertrophy of the LV myocardium.

The last 3 forms are extremely rare and are not accompanied by the development of obstruction of the LV outflow tract.

GENETICS AND MORPHOLOGY

As already mentioned, the disease is transmitted in an autosomal dominant manner. It is based on a mutation in one of the 10 genes encoding sarcomere proteins in the myocardium. As a result, abnormal contractile proteins are found in cardiomyocytes, which allows HCM to be considered a “sarcomere disease.” The synthesis of abnormal proteins is determined by chromosomal point mutations. Proteins whose pathology causes the development of HCM currently include:

Cardiac beta-myosin heavy chain (chromosome 14);

Cardiac troponin T (chromosome 1);

Myosin-associated protein C (chromosome 11);

Tropomyosin (chromosome 15);

Light chain myosin; titin;

Alpha actin;

Cardiac troponin I;

Cardiac alpha-myosin heavy chain.

In total, more than 100 different mutations associated with HCM have been identified: in 10 genes encoding sarcomeric proteins, mutations in 2 genes encoding non-sarcomeric proteins and one mutation in mitochondrial DNA. The most common mutant genes encoding the synthesis of the cardiac myosin heavy chain, cardiac troponin T and myosin-associated protein C. The molecular defects responsible for the development of HCM in unrelated patients are most often different.

Troponin T mutations are associated with increased energy consumption, which predisposes to ischemia and arrhythmias. Some authors have associated troponin T mutations with an increased incidence of VS. Some myosin heavy chain mutations are also associated with an increased risk of arrhythmias and mortality. However, it is still unknown to what extent specific ion current disorders are responsible for electrophysiological abnormalities and what role widespread fibrosis plays. It is assumed that in HCM there is a genetically determined defect in the adrenergic receptor apparatus or an anomaly in the transmembrane transport of calcium ions with increased entry into the cell and sensitization of the myocardium to the action of catecholamines.

Obviously, not all people carrying the genetic mutation develop the phenotypic features of HCM. There is a point of view that the phenotypic manifestations of the disease are changed by modifier genes. For example, functional variants in the angiotensin-1 converting enzyme gene may influence the HCM phenotype and are associated with the risk of VS. Interestingly, the clinical manifestations of diastolic dysfunction can be detected earlier than echocardiography signs of the disease.

It is assumed that the phenotype of the disease is formed as the body grows and develops, and morphological manifestations are revealed upon reaching 17-18 years of age. The absence of signs of the disease in young and even middle age in the presence of a family history of the disease cannot guarantee the absence of HCM in the patient.

It is recommended to screen first-degree relatives. Moreover, if genetic analysis is not possible, an annual examination is recommended, including a clinical examination, 12-lead ECG, echocardiography up to the age of 18. Further, it is recommended to conduct examinations every five years.

Screening in children under 12 years of age is not advisable unless there is a high familial risk and serious sports participation is planned.

Microscopic changes in the myocardium in HCM are characterized by hypertrophy of muscle fibers and disruption of their mutual orientation; the fibers are randomly located at an angle to each other, intersect or form vortices (Fig. 2.2). Were

Rice. 2.2. The location of myocytes is normal (a) and in patients with HCM (b)

Significant disturbances in the distribution of intercellular connections were revealed in areas with a disrupted myofibril structure - connections were located over the entire surface of the cell, whereas normally they are concentrated in well-defined intercalary discs.

This random arrangement of fibers creates a morphological substrate for the occurrence of circulation and re-entry of the excitation wave (re-entry), predetermining the occurrence of paroxysmal rhythm disturbances.

Hypertrophy of myocardial fibers is accompanied by accumulations of mitochondria and glycogen, as well as perinuclear clearings of the cytoplasm (“halos”). The observed degenerative changes, sometimes thickening of the endocardium and interstitial fibrosis, are secondary. In some cases, flat fibrous plaques are found on the endocardium of the thickened part of the interventricular septum, which form at the site of repeated contact with the anterior leaflet of the mitral valve.

Findings characteristic of HCM are considered to be significantly pronounced disturbances in the normal mutual orientation of muscle fibers and fibrositis of the endocardium of the interventricular septum. The remaining microscopic changes are nonspecific and can be detected with myocardial hypertrophy of any origin. The epicardial coronary arteries are not changed in HCM.

HCM is based on a mutation in one of the 10 genes encoding sarcomere proteins in the myocardium.

HEART GEOMETRY AND FEATURES

HEMODYNAMICS

Myocardial hypertrophy in HCM is often most pronounced in the area of the interventricular septum (IVS), that is, it is asymmetric and is accompanied by disorganization of myocytes and myofibrils (Fig. 2.3). In addition, the disease is associated with the development of myocardial fibrosis and damage to small vessels.

The consequence of changes in LV geometry and myocardial morphology in HCM is primarily a violation of LV diastolic filling, which leads to shortness of breath and other manifestations of heart failure, which is one of the causes of death in patients. Syncope associated with the development of ventricular tachyarrhythmias is also characteristic. The latter are a common cause of VS in patients with HCM.

In 20% of cases, a dynamic gradient is formed in the LV outflow tract, caused by a combination of increased flow velocities and narrowing of the outflow tract due to anterior systolic movement of the mitral valve (ASMV).

Externally, the heart is slightly changed. There is LV hypertrophy without dilatation of its cavity; at the same time, the left atrium may be dilated, which indicates a violation of the diastolic filling of the ventricle.

The gradient in the left ventricular outflow tract (LVOT) produces a loud systolic murmur. In addition, hypertrophy of the basal part of the IVS and a narrow outflow tract are detected, as well as, in many patients, thickened and elongated mitral valve leaflets.

Obstruction of the LV outflow tract (LVOT). As follows from the classification, not all patients with HCM have narrowing in the LV outflow tract. Moreover, in most patients there is no gradient in the LVOT at rest. The division of patients into groups with and without LV outflow tract obstruction is extremely important from a clinical point of view. This is due to the fact that almost all medical and surgical treatment strategies for HCM are aimed primarily at patients with signs of LVOT obstruction. The choice of drugs and methods of surgical correction is determined by the hemodynamic status of the patient.

Subaortic steno h is a consequence of the anterior systolic movement of the anterior valvular leaflet (APMVK) and the resulting

its mid-systolic contact with the IVS. Increased contractility of the LV in combination with a slight narrowing of the outflow tract by the subaortic muscular ridge leads to accelerated expulsion of blood into the aorta. An increase in the linear speed of the blood stream causes the Venturi effect - the “suction” effect of a fluid flow moving at a high speed, which draws the anterior leaflet of the mitral valve into the outflow tract of the LV, leading to its significant narrowing and the appearance of a pressure gradient. In some cases, the anterior mitral valve leaflet (AMV) touches the IVS myocardium. Obstruction, thus, occurs only during the second half of the expulsion period, and its severity increases with increasing LV myocardial contractility (Fig. 2.3).

Rice. 2.3. EchoCG of a patient with HCM in M-mode; anterior systolic movement of the mitral valve apparatus is visible (marked by an arrow)

Anterior systolic movement of the MV not only leads to LVOT obstruction, but also to the occurrence of mitral regurgitation (MR) of varying severity. In this case, the direction of the regurgitation jet is characteristic of the posterior wall of the LV. The regurgitation jet directed towards the center and the anterior wall of the atrium, as well as the presence of several flows, require the exclusion of independent pathology of the mitral valve.

In approximately 5% of cases, the presence of a gradient and reduced output is caused primarily by myocardial hypertrophy in the central

ny part of the LV cavity and changes in the anterolateral papillary muscle in the absence of contact of the PMC with the IVS.

The subaortic gradient (more than 30 mm Hg or more) and the associated increase in intraventricular pressure are extremely important both from the point of view of the pathophysiology of the disease and from the point of view of the prognosis of patients. LVOT obstruction is an independent predictor of death due to HCM, the development of severe heart failure (NYHA class III-IV), as well as death from heart failure and stroke. At the same time, an increase in the gradient of more than 30 mm Hg. according to the researchers, is not accompanied by an additional increase in risk.

It is now generally accepted that obstruction in HCM is dynamic. It is recommended to divide all patients with HCM into “hemodynamic subgroups” according to the level of the peak gradient in the LVOT, changed using continuous wave Doppler:

1. The gradient at rest is equal to or greater than 30 mmHg. (2.7 m/s, according to Doppler ultrasound);

2. Latent gradient - at rest less than 30 mm Hg. and increases to 30 or more when conducting provocative tests;

3. Non-obstructive cardiomyopathy - gradient below 30 mmHg. at rest and under provocation.

A number of provocative tests have been proposed to simulate the load during echocardiography (as well as during cardiac catheterization). These include medicinal tests with amyl nitrite, isoproterenol, dobutamine, as well as mechanical tests - the Valsalva maneuver, orthostasis and physical activity. The most physiological and common is to conduct a treadmill test or bicycle ergometry in parallel with Doppler echocardiography.

In addition to obstructive disorders, intracardiac hemodynamics in HCM has the following features:

1. High levels of systolic function (small end-diastolic volume, high ejection fraction and ejection rate), due to increased contractility of the hypertrophied myocardium;

2. Impaired diastolic function due to increased rigidity of the hypertrophied myocardium, which disrupts its diastolic relaxation and makes it difficult to fill the ventricular cavity with blood during diastole;

3. Frequent presence of MR, the main causes of which are:

Displacement of the anterior papillary muscle closer to the mitral valve, which occurs as the size of the cavity decreases

Fibrous changes in the anterior mitral leaflet that occur when it is hemodynamically damaged by accelerated turbulent flow in the outflow tract or contact with the IVS;

Secondary lesions of the mitral valve (calcification, bacterial endocarditis), leading to its dysfunction.

The consequence of changes in the geometry of the LV myocardium in HCM is a violation of LV diastolic filling, as well as syncope associated with ventricular tachyarrhythmias. Due to the anterior systolic movement of the anterior mitral valve leaflet and its contact with the IVS, a dynamic gradient in the LV outflow tract can be created.

CLINIC

The clinical picture of HCM is highly variable - from asymptomatic forms to severe clinical manifestations or sudden death. The diagnosis is facilitated by a family history of cases of HCM or VS. In the absence of complaints, the first manifestation of the disease is usually the detection of systolic murmur or ECG changes (signs of LV hypertrophy). The first complaints usually appear at the age of 20-25 years.

The characteristic clinical picture of HCM is represented by a triad:

Angina;

Arrhythmias;

Syncope.

A typical combination of these symptoms is a systolic murmur, ECG changes, and indications of a history of HCM or VS among relatives.

Chest pain due to HCM often have a typical anginal character caused by myocardial ischemia. They represent a classic example of angina syndrome not associated with coronary artery occlusion. Atypical pain is less common. Myocardial ischemia in HCM is associated with relative coronary insufficiency. Since the reserves for increasing coronary blood supply are not unlimited, a discrepancy arises between the maximum

but possible coronary blood flow and increased need of hypertrophied myocardium for arterial blood. However, in older age groups, in order to confidently exclude a combination with CAD, it is necessary to perform coronary angiography.

A characteristic symptom of HCM is syncope and presyncope, which causes severe weakness, dizziness, and darkening of the eyes. They may be caused by a decrease in cardiac output and insufficient blood supply to the brain as a result of obstruction of the LV outflow tract or episodes of tachyarrhythmias.

Arrhythmic syndrome occupies an important place in the clinical picture of HCM and largely determines the prognosis of the disease. It is generally accepted that the substrate for the development of arrhythmias is a combination of hypertrophy, disruption of myocyte structure, fibrosis and abnormal distribution of discontinuous intercellular connections (connexons). It is assumed that in areas with a disrupted myofibril structure, anisotropy changes and abnormal impulse conduction occurs, possibly leading to the development of re-entry.

Patients complain of sudden attacks of palpitations of varying duration, although short episodes may be asymptomatic. Therefore, to identify rhythm disturbances, Holter ECG monitoring is recommended for all patients with HCM.

The range of detectable arrhythmias is extremely wide. Most of them are ventricular arrhythmias of various gradations - from single extrasystoles and short runs of ventricular tachycardia of 3-5 contractions, not always felt by patients, to life-threatening paroxysms of bidirectional tachycardia with the possibility of developing ventricular fibrillation and sudden death. Paroxysms of supraventricular tachycardia and atrial fibrillation or flutter are also noted. The appearance of a permanent form of atrial fibrillation is a prognostically unfavorable sign, often preceding the development of congestive NK, and is a risk factor for embolic complications. WPW syndrome is often observed.

Heart failure. LV diastolic dysfunction is one of the main hemodynamic manifestations of the disease and sometimes appears even before the detection of typical changes on echocardiography. Inspiratory dyspnea may occur with HCM

one of the earliest symptoms. Its occurrence is associated with a violation of the diastolic filling of the LV, resulting in stagnation in the pulmonary circulation. Hepatomegaly and other congestion in the systemic circulation are observed rarely, mainly in the terminal stage of the disease.

External examination data for HCM meager. Patients usually have a regular physique and well-developed muscles; pallor and cyanosis are absent. An increase in heart size cannot always be detected. The apical impulse is strengthened, sometimes shifted to the left. In the obstructive form of HCM, there may be characteristic physical symptoms:

intermittent jerky pulse;

Increased contractions of the left atrium palpated in the precordial region;

Systolic murmur.

Systolic murmur is heard at the apex and in the third or fourth intercostal space along the left edge of the sternum. The noise has a blowing character and can be carried out in the axillary region. The noise is somewhat distant from the first tone.

Functional and pharmacological tests are of great importance for interpreting the auscultatory picture. The intensity of systolic murmur directly depends on the magnitude of the pressure gradient in the outflow tract from the ventricle. Therefore, all effects that reduce preload and diastolic filling of the LV lead to increased myocardial contractility, cause an increase in the degree of obstruction and, consequently, an increase in noise and its earlier appearance. Such changes occur during the transition to orthostasis, Valsalva maneuver, taking vasodilators (nitroglycerin), during tachycardia. Physical activity, taking digoxin or isadrin also increase the contractility of the LV myocardium and lead to increased noise.

Moving to a horizontal position, squatting, and taking beta-blockers have the opposite consequences. In these tests, bradycardia and increased venous return to the heart increase LV filling in diastole, which reduces the systolic flow rate and the intensity of the systolic murmur.

The characteristic clinical picture of HCM is represented by a triad: angina pectoris, arrhythmias, syncope.

Angina pectoris, as a rule, is not associated with the presence of stenotic plaques in the coronary arteries, but this cannot be excluded in older age groups.

Course and prognosis. The prognosis for HCM is considered unfavorable, with a mortality rate of up to 5% per year. More than half of the deaths, mainly among young patients, are cases of VS. The risk of arrhythmogenic VS especially increases during physical activity or sports.

Unfavorable prognostic factors are:

large mass of LV myocardium;

Ventricular arrhythmias of high grades according to Lown;

Syncope;

Cases of VS among relatives.

Among older patients with HCM (over 40 years), the cause of death is more often congestive NK and the addition of infective endocaditis.

Important factors determining prognosis and choice of therapy are:

1. High risk of VS (taking into account medical history);

2. Progression of symptoms, such as chest pain, shortness of breath and impaired consciousness (syncope, presyncope, dizziness) with preserved LV systolic function;

3. Progression of heart failure with the development of LV remodeling and systolic dysfunction;

4. Complications associated with the development of atrial fibrillation, including embolic stroke.

It must be remembered that HCM is often combined with other heart diseases, such as arterial hypertension and coronary artery disease, which may affect the choice of therapy.

The prognosis for HCM is relatively poor. More than half of deaths are sudden due to ventricular tachyarrhythmias, the risk of which increases during physical activity, especially of a sports nature.

INSTRUMENTAL DIAGNOSTICS

ECG. In patients with HCM, the ECG usually shows a picture of LV myocardial hypertrophy with asymmetrical inverted

teeth T and obliquely downward depression of the segment ST(Fig. 2.4). Severe hypertrophy of the IVS leads to the appearance of pathological teeth Q, which can lead to erroneous diagnosis of AMI. Pathological teeth Q with HCM, they are usually deep, but not widened and pointed, recorded in leads II, III, avF, in the left chest leads, sometimes in leads V3-V4. At the same time, the amplitude of the teeth may decrease R in leads V2-V4. Signs of atrial hypertrophy are often recorded in the form of enlargement and splitting of the teeth PI, II.

Rice. 2.4. ECG of a patient with HCM. Signs of LV hypertrophy

In the apical form of HCM, its only manifestation may be ECG changes in the form of a picture of LV myocardial hypertrophy with giant negative T waves in the precordial leads, the depth of which can exceed 10-15 mm.

Chest X-ray for HCM is not very informative. The cardiac shadow is unchanged or demonstrates left ventricular enlargement (Fig. 2.5) Dilatation of the ascending aorta may be detected. Cardiomegaly is rarely detected, and in HCM it can be caused by myocardial hypertrophy without dilatation of the LV cavity.

Less common radiographic symptoms include:

Moderate enlargement of the left atrium (especially in the presence of mitral regurgitation);

Slightly pronounced congestion in the pulmonary circulation;

Calcification of the mitral valve annulus fibrosus (which may lead to an erroneous diagnosis of rheumatic heart disease).

With angiocardiography a decrease in the LV cavity and its deformation in the form of an “hourglass” during systole is detected, but the need for this study usually arises only in preparation for surgical treatment. At the same time, during cardiac catheterization, pressure gradients along the outflow tract from the LV are determined.

EchoCG is the most valuable and informative non-invasive test for HCM.

Classic echocardiographic signs of HCM are:

1. Asymmetric thickening of the IVS, in which the ratio of the thickness of the septum to the thickness of the opposite part of the posterior wall of the LV is 1.3 or more (up to 2.5-3.0) (Fig. 2.5);

2. Hypokinesia of the IVS (the amplitude of its displacement during cardiac contraction is less than 3 mm);

3. Reduction of the LV cavity, especially pronounced during systole. Due to powerful muscle contraction of the hypertrophied myocardium, closure of the walls of the ventricle can occur, during which complete disappearance (elimination) of its cavity is observed;

4. Significant increase in LV contractility indicators, among which the increase in EF is especially pronounced;

5. Expansion of the cavity of the left atrium.

In the presence of a pressure gradient in the LV outflow tract, the following phenomena are additionally noted:

Rice. 2.5. X-ray of a patient with HCM

1. Anterior systolic movement of the mitral valve - displacement of the anterior mitral leaflet forward during systole and bringing it closer to the IVS - up to touching (Fig. 2.6, see inset);

2. Systolic covering of the aortic valve - some convergence of the aortic leaflets in mid-systole due to a decrease in the rate of blood expulsion from the LV with the development of dynamic obstruction; at the end of the ejection period, additional opening of the valve leaflets may be observed.

Doppler echocardiography allows you to non-invasively detect:

1. Pressure gradient along the LV outflow tract (at rest and/or during provocation) (Fig. 2.7);

2. Mitral regurgitation;

3. Signs of impaired diastolic relaxation of the LV myocardium.

A special place in the diagnosis of this disease is played by echocardiography using provocative tests, which was mentioned above.

Classic EchoCG signs of HCM are: asymmetric thickening of the IVS, its hypokinesia, reduction of the LV cavity, a significant increase in LV contractility, expansion of the left atrium cavity. In the presence of a pressure gradient in the LV outflow tract, anterior systolic movement of the anterior mitral valve leaflet and closure of the aortic valve are noted.

DIFFERENTIAL DIAGNOSIS

The main difficulties usually arise in the differential diagnosis of HCM with valvular AS and CAD.

The similarity of the picture of HCM and AS is due to the presence of systolic murmur, signs of LV hypertrophy, angina syndrome, and small output syndrome.

In AS, there is usually severe valve calcification and weakened pulsations in the peripheral arteries. The systolic murmur is heard at the base of the heart with a maximum in the second intercostal space to the right of the sternum, has a rough, scraping character and is well transmitted to the vessels of the neck. The magnitude of the pressure gradient and intensity

The noise intensity changes little during functional tests.

EchoCG in patients with AS reveals:

Coarse calcification and thickening of the aortic valves;

Their fusion along the commissures;

Significant limitation of systolic opening;

Aortic regurgitation of varying degrees is often present.

In rheumatic aortic disease, changes usually affect the mitral valve - even in the absence of a formed mitral valve, previous valvulitis is indicated by small commissural adhesions that pull the posterior mitral leaflet to the anterior one, or compaction and calcification of the free edges of the leaflets.

With the calcifying type of AS, significant calcification of the mitral apparatus is usually also found, mainly the base of the posterior mitral leaflet, the heads of the papillary muscles and chordae tendineae.

The complexity of the differential diagnosis of HCM with CAD is associated with the commonality of the main clinical syndrome - angina pectoris.

CHD is characterized by a later onset of clinical symptoms of the disease - after 40 years. A systolic murmur can be heard, but it is caused by mitral regurgitation, therefore it is localized at the apex, carried to the axillary region, has a decreasing character and begins immediately after the first sound.

Stress echocardiography and radionuclide ventriculography reveal segmental disorders of myocardial contractility. In doubtful cases, coronary angiography is necessary.

Asymmetric thickening of the IVS and EchoCG signs of outflow tract obstruction, similar to those present in patients with HCM, can occasionally be observed with LV myocardial hypertrophy of secondary origin - in arterial hypertension, in patients with chronic renal failure or in athletes. In such cases, differential diagnosis with HCM presents significant difficulties, since the possibility of a combination of the two conditions cannot be excluded. Of great importance in establishing the correct diagnosis is the study of family history, the presence of syncope and the identification of arrhythmic syndrome.

The main difficulties usually arise in the differential diagnosis of HCM with AS and CAD.

TREATMENT

The choice of treatment strategy for HCM is based primarily on the presence or absence of LV outflow tract obstruction, as well as the predominance of certain symptoms of the disease caused by pathological myocardial hypertrophy (angina pectoris, shortness of breath, syncope, etc.).

Pharmacotherapy

The main directions of drug treatment for HCM are:

1. Reducing the degree of obstruction of the LV outflow tract;

2. Antiarrhythmic therapy;

3. Reducing myocardial oxygen demand.

The main place in the treatment of HCM is occupied by beta-blockers, verapamil, disopyramide.

Beta blockers- drugs with negative inotropic and antiarrhythmic effects, and therefore their use is justified in patients with HCM both in the presence and absence of LVOT obstruction.

The use of β-blockers significantly reduces the severity of all clinical manifestations of the disease, mainly angina and syncope. A decrease in the degree of obstruction of the LV outflow tract under the influence of beta-blockers is caused by a decrease in myocardial contractility as a result of direct negative inotropic action and a decrease in heart rate, which prolongs the period of diastolic filling of the LV and increases its end-diastolic volume.

The antiarrhythmic effect of β-blockers is manifested in a decrease in the frequency of recorded arrhythmic episodes, but the likelihood of VS does not decrease.

The first drug for the treatment of HCM was propranolol, and later more selective drugs (metoprolol, nadolol) were used. Currently, there are many studies indicating an improvement in the clinical status of patients with HCM

against the background of the use of drugs of this group. Therapy with beta-blockers begins with small doses and then increases them to those required to control symptoms (within clinically acceptable limits). The daily dose may vary between patients and as the disease progresses. Propranolol doses of 240–480 mg in adults have been shown to be effective in HCM in numerous studies. Some studies have attempted to prescribe ultra-high doses of propranolol (up to 1000 mg/day), but this tactic has not been widely used.

Verapamil. It has been used to treat patients with HCM since 1979 and is recommended for the treatment of patients with both LVOT obstruction and non-obstructive forms.

Verapamil at a dose of up to 480 mg/day helps to reduce the severity of symptoms, which is likely due to both a negative inotropic effect and a decrease in heart rate, as well as improved LV relaxation. However, there is evidence of the possibility of side effects of verapamil in patients with obstructive HCM. The negative hemodynamic effects of verapamil, including cases of worsening LVOT obstruction, pulmonary edema and cardiogenic shock, are associated with the fact that vasodilation during its use prevails over the negative inotropic effect. Therefore, caution when using verapamil should be observed primarily in patients with severe LVOT obstruction, not indicated for children.

International recommendations do not indicate the possibility and safety of using the drug diltiazem in patients with HCM. Nifedipine and other dihydropyridine derivatives, which have a mainly arteriolodilating effect, significantly reduce afterload, so their use in HCM is not recommended and can lead to an increase in obstruction.

It is currently believed that for patients with HCM with outflow tract obstruction and ventricular arrhythmias, the first choice drugs are definitely beta-blockers. At the same time, in patients with predominant diastolic myocardial dysfunction without significant obstructive disorders and with supraventricular arrhythmias, non-dihydropyridine calcium antagonists can be used. The combination of drugs from these groups seems inappropriate due to the risk of severe bradycardia and the development of AV block.

In obstructive HCM, even in the presence of congestive NK, the administration of cardiac glycosides, nitrates and other peripheral vasodilators, ACE inhibitors and diuretics is not recommended, since their use leads to worsening obstruction of the LV outflow tract.

An exception can be made for cardiac glycosides when a tachysystolic form of atrial fibrillation occurs.

Disopyramide(ritmilen, rhythmodan) is a class 1a (quinidine-like) antiarrhythmic drug. There are indications of a decrease in the severity of symptoms in patients with LVOT obstruction at rest. It is believed that the use of the drug helps to reduce the amplitude of the anterior systolic movement of the PMC and the volume of mitral regurgitation. In addition, it has a negative inotropic effect on the myocardium. In patients with HCM, it is used for life-threatening ventricular arrhythmias. The initial daily dose is 200-300 mg (depending on body weight), maintenance - 300-600 mg/day. in 4 doses. The use of the drug requires interval control QT.

Amiodarone(cordarone) is not included in international recommendations for the treatment of this disease. They are used for HCM as an antiarrhythmic agent when beta blockers and verapamil are ineffective.

The appointment of cordarone is indicated for patients who have the following rhythm disturbances recorded on ECG or Holter monitoring:

1. Life-threatening ventricular arrhythmias or their precursors (frequent polytopic ventricular extrasystoles, paired ventricular extrasystoles, recurrent paroxysms of ventricular tachycardia, episodes of ventricular fibrillation);

2. Paroxysms of supraventricular tachycardia;

3. Paroxysms of atrial fibrillation or flutter. The initial (loading) dose of amiodarone is 800-1000

mg/day (rarely - up to 1600 mg / day), divided into 2-4 doses, for 1-3 weeks. Maintenance dose - 100-400 mg/day. in 1-2 doses.

Amiodarone can be used together with beta-blockers (with caution, with careful monitoring of pulse rate, ECG and blood pressure levels), but its combination with calcium antagonists is contraindicated.

Contraindications to the use of amiodarone are:

Sinus bradycardia;

AV conduction disorders;

Low LV ejection fraction (less than 40%);

Lengthening the Q-Γ interval.

In some patients, drug therapy does not achieve stabilization and reduction in the severity of symptoms.

The main place in the pharmacotherapy of HCM is occupied by beta blockers, verapamil, disopyramide.

Surgery

Data from various researchers on the need for patients to undergo surgical treatment varies and ranges from 5 to 30%.

The basis for deciding on surgical correction are:

1. Reliable detection of a high gradient in the LVOT (>50 mmHg);

2. Severe symptoms (HF, angina), refractory to maximum drug therapy.

The complexity of the anatomical substrate that causes obstruction in HOCM is reflected in the large number of different surgical techniques that have been introduced over the past 30 years.

Myotomy-myectomy

The most widespread operation is to cut and remove overgrown muscle tissue. (myectomy) in the basal part of the IVS, also known as the Morro procedure. In some cases, an operation is performed to resection the hypertrophied part of the IVS with simultaneous plication of the PMC or mitral valve replacement; in some cases, to increase the volume of the cavity, resection of the papillary muscles is performed. Most observations show the effectiveness of LV myotomy-myectomy in reducing the gradient in the LVOT and improving clinical manifestations. The transaortic approach is usually preferred for left- and right-sided or combined ventriculotomy.

Early studies using this technique were marred by the high risk of surgical mortality (10–15%). These figures are usually cited by proponents of alternative procedures, such as dual chamber stimulation with RV pre-excitation and artificial infarction of the basal IVS. However, as a result of improvements in myocardial protection techniques and preoperative preparation, the perioperative risk for patients with severe hypertrophy has now been significantly reduced.

All patients are operated on using a cardiopulmonary bypass with cardiac arrest and moderate hypothermia (32 °C). In the case of concomitant CABG, coronary anastomoses are performed first. All other concomitant surgical interventions are performed after completion of extended myectomy and IVS repair.

Quite often it is complicated by the development of left bundle branch block or complete transverse block. In addition, complications include perforation of the IVS during the intervention and insufficient volume of surgery due to the limited exposure area. Most modifications are based on the development of techniques for expanded access to the obstruction substrate

Extended myectomy allows access to the deeper structures of the ventricle, where resection of the hypertrophied trabecula and mobilization or partial excision of the papillary muscles leads to correction of the anatomically deformed subvalvular apparatus of the mitral valve. A low transverse aortotomy and incisions are made to the commissure of the aortic valve, which exposes access to the basal parts of the IVS.

The prevalence of hypertrophy is determined visually and by palpation. A sharp three-pronged hook - a retractor - is carefully installed at the deepest point of the hypertrophied septum, thus precisely separating the intraventricular part of the overgrown muscle tissue. The retractor moves forward into the surgeon's field of vision. Thus, the muscle mass intended for resection is clearly recorded and isolated. Longitudinal incisions are made 2-3 cm below the fibrous ring of the AC in the direction of the retractor teeth - the first at the deepest point of the right coronary cusp - directly under the right coronary foramen and the second - in the direction of the free wall of the LV. This incision may even extend to the insertion of the mitral valve.

valve Both incisions are then connected by a transverse incision to completely remove the tissue held by the retractor.

Once a wide and deep LVOT is created, access to the deeper structures of the LV becomes available. Both papillary muscles are then completely mobilized, and all hypertrophied trabeculae, as well as hypertrophied parts of the papillary muscles, are resected. At this stage of the operation, good visualization of the surgical field is necessary to ensure the safety of resection. In cases of significant RVOT obstruction, excess right ventricular tissue is excised through an incision, which is covered with tissue or by applying a patch.

Alcohol-induced septal necrosis

Another method of non-drug treatment of patients with HCM was the creation of alcohol-induced septal necrosis (infarction). A picture of anteroseptal Q-myocardial infarction develops with corresponding ECG changes, increased CPK activity and the occurrence of blockade of the right branch of the His bundle, as a result of which the thickness of the myocardium of the basal part of the septum decreases. However, the incidence of arrhythmic episodes and the risk of sudden death remain the same.

Such therapeutic necrosis leads to a decrease in obstruction of the left ventricular outflow tract and a decrease in the intraventricular gradient, which leads to an objective improvement in the clinical course of the disease.

Unfortunately, there are few data on long-term outcomes. The first successful percutaneous myocardial reduction by injection of a small amount of 96% alcohol into the first major septal branch of the left anterior descending coronary artery (LADCA) was performed in 1994 and reported in 1995.

During recent years, several modifications of the original ablation technique have been proposed. The amount of alcohol used (initially 3-5 ml) has now decreased to 2 ml or less, and the use of control using transesophageal echocardiography has increased the safety of the procedure.

Currently, the septal branch is identified by injecting ultrasound contrast. The penetration of alcohol into the LPNCA is excluded by the forced introduction of 1-2 ml of X-ray

gical contrast into the target vessel through the lumen of the inflated balloon catheter. It is vital to prevent LAD injury and anterior wall infarction. To do this, use a short balloon of adequate size, which is inflated for 5 minutes. and more after the last injection of alcohol.

Resynchronization therapy

Since 1967, it has been known that right ventricular apical electrical stimulation leads to a decrease in the LV outflow tract gradient and a decrease in clinical manifestations. Randomized trials suggest that older patients are optimal candidates for cardiac pacing. To determine the patient's sensitivity to this type of therapy, temporary pacemakers are installed. This type of treatment does not affect myocardial hypertrophy and other causes of the disease. Pacing is a reversible and effective treatment method and poses minimal risk to the patient, and therefore its possibility should be considered in a timely manner among the entire range of therapeutic measures.

Due to its dynamic nature, LVOT obstruction can change markedly due to changes in pre- and afterload and LV contractility. The results of the studies show that electrical stimulation can be effective in patients with a gradient in the LVOT, detected both at rest and during provocation.

On average, the degree of gradient reduction during cardiac stimulation, according to various authors, varies from 30 to 50%, and in some studies it approached 100%.

Reversal of septal motion, which results from early apical stimulation activating the LV apical region before the septal region, is thought to result in an increase in LVOT diameter and is thought to be a key mechanism underlying the decrease in LVOT gradient. Pacing has been shown to reduce contractility, especially in the septal region, and cause desynchronization of LV contraction. This results in a significant increase in LV end-systolic volume and a rightward shift in the pressure-volume curve.

MR is closely associated with LVOT obstruction, and therefore it can be assumed that reducing its obstruction should reduce the severity of

ness of MR. MR, defined as the ratio of the volume of regurgitation to the area of the left atrium, according to modern studies, also decreases after installation of a pacemaker. It is important to note that MR remained unchanged in patients with persistent high gradient or organic mitral valve pathology, which should be considered as a contraindication to pacing.

Data on the effect of pacing on diastolic function are conflicting. Acute impairment of diastolic function was observed in a number of patients, while in other studies there was either no change or improvement in LV diastolic function. Pacing, if successful, should be considered as a long-term treatment option.

Installation of implantable cardioverter-defibrillators. It is generally accepted that the substrate for the development of arrhythmias and VS in HCM is a combination of hypertrophy, disruption of myocyte structure, fibrosis and abnormal distribution of discontinuous intercellular connections (connexons). It has been shown that the magnitude of hypertrophy is directly related to the risk of VS and is an independent prognostic factor. VS in patients with HCM is often associated with physical activity.

For most patients at increased risk of VS, therapy aimed at the leading mechanism is ineffective, and amiodarone and/or an implantable cardioverter defibrillator are added to treatment. Predictors of VS in HCM are a family history of VS in family members under 45 years of age, syncope, especially recurrent and associated with physical activity, severe hypertrophy of the left ventricular myocardium (wall thickness >3 cm), decreased blood pressure in response to physical activity, attacks of unstable ventricular tachycardia during Holter monitoring. A cardioverter-defibrillator must be installed in cases of sustained ventricular tachycardia and in patients who have had a successfully treated episode of VS. The use of a cardioverter defibrillator presents several challenges in children and adolescents related to growth, development, physiological adaptation to the device, and possible modification of the device. The principle of managing such patients is the use of amiodarone as a transitional step to further implantation of a cardioverter-defibrillator.

Hypertrophy of the left or right parts of the heart occurs due to damage to the muscles, valves of the organ, due to disruption of blood flow. This often happens with congenital developmental anomalies, due to increased blood pressure, lung diseases, and significant physical activity. Most often, hypertrophy of the left ventricle of the heart is detected. This is due to the greater functional load in this area.

Reasons for appearance

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Reasons for appearance

The disease occurs as a result of various disorders that interfere with the normal functioning of the organ. The myocardium begins to contract with increased load, its metabolism increases, tissue volume and cell mass increase.

At the initial stage of the disease, the heart maintains normal blood flow due to an increase in its mass. But later the myocardium is depleted, and hypertrophy gives way to atrophy - the cells significantly decrease in size.

There are two types of pathology: concentric - the heart enlarges, its walls thicken, the atria/ventricles decrease, and eccentric (the organ is enlarged, but the cavities are expanded).

Cardiac hypertrophy can affect healthy people involved in physical labor and athletes. Against the background of such changes, acute heart failure may occur. When engaging in bodybuilding, hockey, or heavy physical labor, you need to monitor the condition of the myocardium.

Due to its occurrence, ventricular hypertrophy is divided into 2 types:

working – due to increased load on a healthy body;
substitution is the result of adaptation to work with another disease.

Causes of left ventricular damage

Most often, the muscle of the left ventricle undergoes changes. If its thickness is more than 1.2 cm, this violation occurs. In this case, hypertrophy of the IVS (interventricular septum) of the heart is also observed. In severe cases, the thickness can reach 3 cm and the weight can reach 1 kg.

Poor blood pumping into the aorta is provoked, therefore blood supply throughout the body is disrupted. Weight gain leads to a lack of oxygen and nutrients. As a result, hypoxia and sclerosis occurs.

Causes of changes in the left ventricle: arterial hypertension; cardiomyopathy; narrowing (stenosis) of the aortic valve; increased physical activity; hormonal disorders; obesity; kidney disease with secondary hypertension.

Causes of left atrium damage:

Arterial hypertension;
Hypertrophic cardiomyopathy;
Congenital pathologies of the heart/aorta;
General obesity, especially in children and adolescents;
Stenosis/insufficiency of the aortic or mitral valve.

Causes of right ventricular damage

Changes in the right atrium are usually associated with pulmonary pathologies and disturbances in the pulmonary circulation of blood flow. Blood enters the right atrium through the vena cava from tissues and organs. From there it enters the ventricle through the tricuspid valve and further into the pulmonary artery and lungs.

In the latter, gas exchange occurs. It is for this reason that it disrupts the normal structure of the right sections due to various diseases of the respiratory system.

The main factors provoking atrial hypertrophy of right-sided localization:

Congenital developmental pathologies (eg tetralogy of Fallot, IVS defect);
Chronic obstructive pulmonary diseases, for example, emphysema, pneumosclerosis, bronchial asthma, bronchitis;
Tricuspid valve stenosis/insufficiency, pulmonary valve changes, right ventricular enlargement.

Chronic lung pathology provokes damage to the vessels of the small circle, proliferation of connecting tissues, gas exchange and microcirculation decreases. As a result, blood pressure in the vessels of the lungs increases, so the myocardium begins to contract with greater force, which leads to hypertrophy.

Narrowing or incomplete closure of the tricuspid valve leads to the same disruption of blood flow as in a similar case with mitral pathology.

Causes of changes in the right ventricle: congenital malformations, chronic pulmonary hypertension, narrowing of the pulmonary valve, increased venous pressure with congestive insufficiency.

Hypertrophy of the right ventricle of the heart occurs if the thickness of its wall is more than 3 mm. It leads to expansion of the departments and poor blood circulation. As a result, venous return through the vena cava is disrupted, and stagnation occurs. Patients develop swelling, shortness of breath, bluish skin, and then complaints about the functioning of internal organs.

It should be noted that if the left ventricle is damaged, the left atrium will also be affected. Then the right sections are also subject to changes.

Symptoms of hypertrophy of the left and right ventricle of the heart

When the myocardium of the left half is damaged, the following occur: fainting, dizziness, shortness of breath, arrhythmias, pain in this area, weakness, and fatigue.

When the right half is affected, the following symptoms occur: cough, shortness of breath, difficulty breathing; swelling; cyanosis, pale skin; rhythm disturbance.

How is hypertrophy of both ventricles of the heart diagnosed?

The simplest and at the same time effective methods are ultrasound (US) and echocardiography (ECG). In the process, the thickness of the walls and the size of the organ are determined.

Indirect symptoms of changes detected on the ECG:

When the right sections change, the electrical conductivity changes, the rhythm is disrupted, and a deviation of the electrical axis to the right is observed;
Changes in the left sections are indicated by deviation of the axis to the left, respectively, and voltage signs are recorded.

It is also possible to confirm or refute the diagnosis using the results of a chest x-ray.

Treatment of various forms of cardiac hypertrophy

All efforts to eliminate the disease are directed primarily at the cause that caused it.

For example, if a disorder occurs due to a respiratory disease, the course of treatment is aimed at compensating lung function. Anti-inflammatory therapy is prescribed. Bronchodilators and a number of others are used, depending on the underlying cause.

In case of damage to the left side caused by arterial hypertension, treatment only involves taking antihypertensive medications of different groups, as well as diuretics.

If severe valve defects are detected, they may resort to surgery and even prosthetics.

Treatment of hypertrophy of the left and right ventricle of the heart in all cases of the disease includes eliminating the symptoms of myocardial damage. For this purpose, antiarrhythmic therapy is used, as well as cardiac glycosides.

It is possible that drugs will be prescribed that improve the metabolic process in the heart muscle (eg riboxin, ATP, etc.). Patients are advised to adhere to a special diet, limit fluid and salt intake. In case of obesity, efforts are directed towards normalizing body weight.

In case of congenital heart disease, the pathology is eliminated surgically, if possible. In very severe cases, when the structure is severely damaged and hypertrophic cardiomyopathy develops, the only option is an organ transplant.

As can be judged from the above, the approach to patients is carried out purely individually. Doctors take into account all existing manifestations of organ dysfunction, the general condition of the patient, and the presence of concomitant diseases.

It should be noted that timely detected myocardial pathology can be corrected in the vast majority of cases. If you feel the first alarming symptoms, you should immediately seek advice from a specialist - a cardiologist. After the examination, he will identify the cause of the disease and prescribe adequate treatment.

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Causes

The causes of hypertrophic cardiomyopathy were established after an ultrasound scan of the patients' relatives. It turned out that 65% of members of the same family have similar changes in the heart muscle.

There are 2 forms of the disease based on etiology.

Primary or idiopathic

Primary is the hereditary form of cardiomyopathy. The development of genetics has made it possible to establish the exact gene responsible for the development of the disease in half of the cases. In 50% of families, an exact indication of the altered genes has not been established.

The type of inheritance is autosomal dominant. This means that the disease necessarily manifests itself in the heirs, regardless of the gender of the child. Hypertrophic cardiomyopathy in children occurs with a 50% probability if one of the parents is healthy and the other is a carrier of the mutant gene. If both parents have genetic changes, then the probability reaches 100%.

Scientists believe that gene mutation can occur under the influence of unfavorable conditions in the external environment (smoking, previous infections, radiation) affecting the expectant mother during pregnancy.

Secondary

Secondary changes form after the age of 60 in patients with hypertension who had changes in the structure of muscle tissue in the prenatal period.

It has been established that 1/5 of patients who survive to old age may develop systole weakness and dilation of the left ventricular cavity. In such cases, hypertrophic cardiomyopathy does not differ from the dilated type.

The mechanism of pathology development

As a result of genetic mutations, “incorrect” main protein molecules that ensure the contraction process, actin and myosin, appear in muscle tissue. They do not produce the required amount of calories due to a sharp decrease in the content of necessary enzymes. In 90% of patients, muscle cells lose their direction. In the myocardial tissue, areas incapable of contraction are formed.

In response, other fibers take over the work functions. Their muscle mass increases (hypertrophies) because they have to contract with increased load. The thickness of the left ventricle increases, although no data are available on congenital or acquired defects or hypertension. At the same time, thickening of the interventricular septum occurs. This leads to a narrowing of the blood ejection pathways into the aorta.

Areas of hypertrophy may be localized in patches (usually at the exit to the aorta) or affect a large part of the left ventricle. Less commonly, they spread to the right side of the heart muscle. Damage occurs to the valve leaflets (mitral and aortic), and to the vessels supplying the myocardium.

During diastole, the atria have to work harder to fill the ventricles, because the tissues become dense, hard, and lose elasticity. The pressure in the pulmonary circulation increases.

Increased muscle mass requires more oxygen. The discrepancy between the growth of myocardial demands and capabilities leads to the development of ischemia. This is also facilitated by mechanical compression of the mouth of the left coronary artery.

Types of heart damage

Due to the uniformity and symmetry of the development of areas of myocardial hypertrophy, the following forms are distinguished:

symmetrical (concentric) - the thickness of the walls of the left ventricle increases to the same extent along the anterior, posterior surfaces and in the area of the septum, less often hypertrophy of the right ventricle is added;
asymmetric - areas of thickening form in the upper or lower part of the interventricular septum, it becomes one and a half to three times thicker than the posterior wall of the left ventricle (in a normal heart they are equal), in 2/3 of patients these changes are combined with hypertrophy of the region of the anterior, lateral wall of the left ventricle or apex, without changes in the posterior wall.

Based on the strength of the obstruction to the flow of blood from the left ventricle to the aorta, it is customary to distinguish:

obstructive hypertrophic cardiomyopathy (subaortic or subvalvular) - changes in anatomical relationships create a barrier to blood exit;
non-obstructive - there is no barrier.

Clinical picture

Symptoms of hypertrophic cardiomyopathy first appear between the ages of 20 and 25. The most typical are the following:

Pain behind the sternum of a pressing nature, very similar to angina attacks, has a similar irradiation to the left shoulder, neck, and shoulder blade. Unlike angina pectoris, they are not relieved by medications containing Nitroglycerin. There are atypical pains of aching or stabbing type.

An important sign is increased shortness of breath when changing the horizontal position of the body to a vertical one. Over time, increased shortness of breath leads to cardiac asthma and pulmonary edema.
Arrhythmias, increased heart rate.
Dizziness that reaches the point of fainting is associated with a malnutrition of the brain. It intensifies during physical activity, straining, after a heavy meal, and when getting up quickly.

For hypertrophic cardiomyopathy, a characteristic manifestation is the sudden death of a person (the classification specifies that no more than 1 hour should pass from the moment of loss of consciousness, the case cannot have any signs of violence).

How to identify the disease

Diagnosis of the disease is very difficult. The doctor needs to know the family history (cases of confirmed disease in relatives or sudden death at a young age), the course of pregnancy in the mother, connections with industrial toxic substances, previous infectious diseases, stay in areas with high radiation.

During the examination, the doctor pays attention to the pallor of the skin, cyanosis of the lips and fingers. High or normal blood pressure is recorded.

On auscultation, a characteristic murmur is heard over the projection of the aorta.

In order to exclude possible pathology of the heart and blood vessels, a general analysis of blood, urine, biochemical tests for metabolic products, glucose, and the blood coagulation system are checked.

Additional examination methods

Hardware diagnostics allows you to accurately identify disease problems.

An ECG study records information on disturbed rhythm, hypertrophy of the heart, and the development of blockades.
The phonocardiogram records noise from certain points, which makes it possible to establish a connection between the audible noise and the aorta.
An x-ray shows an increase in the contours of the cardiac shadow, but the size may be normal if hypertrophy develops inside the cavity.
Ultrasound is the main method in making a diagnosis. The size of the heart chambers, wall thickness, condition of the valve apparatus, interventricular septum are assessed, and blood flow disturbances are observed.
Magnetic resonance imaging allows you to obtain a three-dimensional image of the heart, identify obstruction, and the degree of wall thickness.
Genetic research is a method of the future, but it is not yet developed enough.
By inserting catheters into the cavities of the heart, the pressure in the atria and ventricles and the speed of blood flow are studied and measured. The technique allows you to take material for a biopsy.
Coronary angiography of the heart vessels is performed in patients over 40 years of age for differential diagnosis with ischemic lesions of the heart vessels.

A biopsy is permissible only if all other diseases are excluded and there is no help from other diagnostic methods. Under a microscope, altered muscle fibers become visible.

Treatment

Specific elimination of gene mutations has not yet been achieved. Treatment of hypertrophic cardiomyopathy is carried out with medications that affect all aspects of the pathogenesis of the disease.

If signs of the disease are detected, it is necessary to limit physical activity and stop playing sports.

If the patient has any chronic infectious diseases, prophylactic antibiotics are prescribed.

Groups of medications that block adrenergic receptors, calcium antagonists are used, and agents that reduce thrombus formation in the cavities of the heart are added.

Surgical methods

The method of choice for open heart surgery is myotomy - removal of part of the interventricular septum from the inside or through the aorta. The mortality rate of these operations reaches 5%, which is comparable to the overall mortality rate.

A more gentle technique is carried out - concentrated alcohol is injected into the septal area through a puncture of the chest and heart under ultrasound control. Cell death and thinning of the septum are artificially caused. The obstacle to the passage of blood is reduced.

To treat the disturbed rhythm, an electrical stimulator or defibrillator is implanted (depending on the type of disorder).

Modern data suggest that survival after surgical treatment for 10 years is 84%, and with continuous conservative treatment - 67%.

In case of obstruction, an operation is used to replace the mitral valve with an artificial one, this eliminates its contact with the septum and “clears” the passage for blood flow.

Course of the disease

Hypertrophy is possible from the moment of birth. But in most patients it begins to appear during adolescence. Over three years, the thickness of the myocardial wall increases by 2 times. However, no symptoms of the disease are found in 70% of patients. By the age of 18 (less often until 40), the progression of thickening of the heart wall stops.

Subsequently, clinical manifestations develop with an obstructive variant of the pathology. In cases of non-obstructive forms, the course is favorable and is detected by chance during an ECG examination.

The annual incidence of sudden death from hypertrophic cardiomyopathy and its complications among adults is up to 3%, among children – from 4 to 6%. The main cause is considered to be ventricular fibrillation.

What are the possible complications?

Hypertrophic cardiomyopathy does not occur in isolation; the disease affects all aspects of the heart’s activity and causes serious complications.

Arrhythmias and impaired conduction are observed in almost every patient. Depending on the severity, they may come out on top in terms of threat to the patient’s life. They are the direct cause of cardiac arrest or fibrillation.
The addition of infection of the mitral and aortic valves leads to the development of endocarditis with subsequent insufficiency of the valve apparatus.
The separation of a blood clot and the introduction of an embolus into the vessels of the brain (up to 40% of cases), into internal organs, and into the arteries of the extremities occurs with atrial fibrillation, a paroxysmal form.
The development of chronic heart failure is possible during a long course of the disease, when part of the myocardial muscle fibers are replaced by scar tissue.

Forecast

Treatment can lead to temporary stabilization of hypertrophy. Average life expectancy does not directly depend on the form of the disease. The most favorable prognosis is considered to be with a long asymptomatic course, as well as with apical localization and the absence of cases of sudden death among relatives.

The main sign that aggravates the prognosis among patients from 15 to 50 years old is considered to be fainting, ischemia, and ventricular tachycardia detected on the ECG. The appearance of shortness of breath and chest pain in a patient sharply increases the risk of sudden death.

Statistical studies show, from the moment of detection, a five-year survival rate of 82 to 98%, a ten-year survival rate of 64 to 89%, with an average annual mortality rate of 1%.

Complexities in the etiological factors of the disease make any kind of prevention almost impossible. With this pathology, the main attention should be paid to identifying it, starting from adolescence, and carrying out symptomatic therapy.

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What is myocardial hypertrophy

This autosomal dominant disease is characterized by hereditary traits of gene mutation and affects the heart. It is characterized by an increase in the thickness of the walls of the ventricles. Hypertrophic cardiomyopathy (HCM) has a classification code according to ICD 10 No. 142. The disease is often asymmetric, with the left ventricle of the heart more susceptible to damage. This happens:

chaotic arrangement of muscle fibers;
damage to small coronary vessels;
formation of areas of fibrosis;
obstruction of blood flow - an obstacle to the ejection of blood from the atrium due to displacement of the mitral valve.

With heavy loads on the myocardium caused by diseases, sports, or bad habits, the body’s protective reaction begins. The heart needs to cope with increased work volumes without increasing the load per unit of mass. Compensation begins to occur:

increased protein production;
hyperplasia – increase in the number of cells;
increase in myocardial muscle mass;
wall thickening.

Pathological myocardial hypertrophy

With prolonged work of the myocardium under loads that are constantly increased, a pathological form of HCM occurs. A hypertrophied heart is forced to adapt to new conditions. Myocardial thickening occurs at a rapid pace. In this situation:

growth of capillaries and nerves lags behind;
blood supply is disrupted;
the influence of nervous tissue on metabolic processes changes;
myocardial structures wear out;
the ratio of myocardial sizes changes;
systolic and diastolic dysfunction occurs;
repolarization is disrupted.

Myocardial hypertrophy in athletes

Abnormal development of the myocardium—hypertrophy—occurs unnoticed in athletes. During high physical activity, the heart pumps large volumes of blood, and the muscles, adapting to such conditions, increase in size. Hypertrophy becomes dangerous, causing stroke, heart attack, sudden cardiac arrest, in the absence of complaints and symptoms. You should not suddenly stop training to avoid complications.

Sports myocardial hypertrophy has 3 types:

eccentric - muscles change proportionally - typical for dynamic activities - swimming, skiing, long-distance running;
concentric hypertrophy - the ventricular cavity remains unchanged, the myocardium increases - observed in gaming and static types;
mixed - inherent in activities with the simultaneous use of immobility and dynamics - rowing, cycling, skating.

Myocardial hypertrophy in a child

It is possible that myocardial pathologies may appear from the moment of birth. Diagnosis at this age is difficult. Hypertrophic changes in the myocardium are often observed during adolescence, when cardiomyocyte cells actively grow. Thickening of the anterior and posterior walls occurs until the age of 18, then stops. Ventricular hypertrophy in a child is not considered a separate disease - it is a manifestation of numerous ailments. Children with HCM often have:

heart disease;
myocardial dystrophy;
hypertension;
angina pectoris.

Causes of cardiomyopathy

It is customary to distinguish between primary and secondary causes of hypertrophic development of the myocardium. The first ones are influenced by:

Secondary causes of myocardial hypertrophy are provoked by the following factors:

Left ventricular hypertrophy

More often, the walls of the left ventricle are susceptible to hypertrophy. One of the causes of LVH is high pressure, which forces the myocardium to work at an accelerated rhythm. Due to the resulting overloads, the left ventricular wall and IVS increase in size. In this situation:

the elasticity of the myocardial muscles is lost;
blood circulation slows down;
normal heart function is disrupted;
there is a danger of a sudden load on it.

Left ventricular cardiomyopathy increases the heart's need for oxygen and nutrients. Changes in LVH can be noticed during instrumental examination. Low output syndrome appears - dizziness, fainting. Among the signs accompanying hypertrophy:

Right atrial hypertrophy

Enlargement of the wall of the right ventricle is not a disease, but a pathology that appears when there is overload in this department. It occurs due to the receipt of a large amount of venous blood from large vessels. The cause of hypertrophy can be:

birth defects;
atrial septal defects, in which blood enters the left and right ventricles simultaneously;
stenosis;
obesity.

Right ventricular hypertrophy is accompanied by symptoms:

hemoptysis;
dizziness;
night cough;
fainting;
chest pain;
shortness of breath without exertion;
bloating;
arrhythmia;
signs of heart failure - swelling of the legs, enlarged liver;
malfunction of internal organs;
cyanosis of the skin;
heaviness in the hypochondrium;
dilation of veins in the abdomen.

Hypertrophy of the interventricular septum

One of the signs of the development of the disease is hypertrophy of the IVS (interventricular septum). The main cause of this disorder is gene mutations. Hypertrophy of the septum provokes:

Dilation of the heart chambers

Hypertrophy of the interventricular septum can provoke an increase in the internal volume of the heart chambers. This expansion is called myocardial dilatation. In this position, the heart cannot perform the function of a pump, and symptoms of arrhythmia and heart failure occur:

fast fatiguability;
weakness;
dyspnea;
swelling of the legs and arms;
rhythm disturbances;

Cardiac hypertrophy - symptoms

The danger of myocardial disease in asymptomatic progression for a long time. It is often diagnosed accidentally during medical examinations. As the disease progresses, signs of myocardial hypertrophy may be observed:

Forms of cardiomyopathy

It should be noted that the disease is characterized by three forms of hypertrophy, taking into account the systolic pressure gradient. All together the obstructive form of HCM corresponds. Stand out:

basal obstruction – resting state or 30 mm Hg;
latent – calm state, less than 30 mm Hg – it characterizes the non-obstructive form of HCM;
labile obstruction – spontaneous intraventricular gradient fluctuations.

Myocardial hypertrophy - classification

For convenience of work in medicine, it is customary to distinguish between the following types of myocardial hypertrophy:

obstructive – at the top of the septum, over the entire area;
non-obstructive – symptoms are mild, diagnosed by chance;
symmetrical – all walls of the left ventricle are affected;
apical - the heart muscles are enlarged only from above;
asymmetrical - affects only one wall.

Eccentric hypertrophy

With this type of LVH, the ventricular cavity expands and at the same time a uniform, proportional compaction of the myocardial muscles occurs, caused by the growth of cardiomyocytes. With a general increase in heart mass, the relative thickness of the walls remains unchanged. Eccentric myocardial hypertrophy can affect:

interventricular septum;
top;
side wall.

Concentric hypertrophy

The concentric type of disease is characterized by maintaining the volume of the internal cavity while increasing the mass of the heart due to a uniform increase in wall thickness. There is another name for this phenomenon - symmetrical myocardial hypertrophy. The disease occurs as a result of hyperplasia of myocardiocyte organelles, provoked by high blood pressure. This development of events is typical for arterial hypertension.

Myocardial hypertrophy – degrees

To correctly assess the patient’s condition with HCM, a special classification has been introduced that takes into account myocardial thickening. According to how much the size of the walls increases during heart contraction, cardiology distinguishes 3 degrees. Depending on the thickness of the myocardium, the stages are determined in millimeters:

moderate – 11-21;
average – 21-25;
pronounced – over 25.

Diagnosis of hypertrophic cardiomyopathy

At the initial stage, with a slight development of wall hypertrophy, it is very difficult to identify the disease. The diagnostic process begins with interviewing the patient, finding out:

presence of pathologies in relatives;
the death of one of them at a young age;
past diseases;
fact of radiation exposure;
external signs during visual inspection;
blood pressure values;
indicators in blood and urine tests.

A new direction is being used – genetic diagnosis of myocardial hypertrophy. The potential of hardware and radiological methods helps to establish the parameters of HCM:

ECG - determines indirect signs - rhythm disturbances, hypertrophy of sections;
X-ray - shows an increase in the contour;
Ultrasound – assesses myocardial thickness, blood flow disturbances;
echocardiography – records the location of hypertrophy, diastolic dysfunction;
MRI – gives a three-dimensional image of the heart, determines the degree of myocardial thickness;
ventriculography – examines contractile functions.

How to treat cardiomyopathy

The main goal of treatment is to return the myocardium to its optimal size. Activities aimed at this are carried out in a comprehensive manner. Hypertrophy can be cured when diagnosed early. An important part in the system of myocardial health is played by lifestyle, which implies:

Drug treatment of hypertrophic cardiomyopathy includes the use of drugs that:

reduce blood pressure - ACE inhibitors, angiotensin receptor antagonists;
regulate heart rhythm disturbances - antiarrhythmics;
drugs with a negative ionotropic effect relax the heart - beta blockers, calcium antagonists from the verapamil group;
remove fluid - diuretics;
improve muscle strength - ionotropes;
if there is a threat of infective endocarditis, antibiotic prophylaxis.

An effective method of treatment that changes the course of excitation and contraction of the ventricles is dual-chamber pacing with a shortened atrioventricular delay. More complex cases - severe asymmetric hypertrophy of the IVS, latent obstruction, lack of effect of the drug - require the participation of surgeons for regression. Help save a patient's life:

installation of a defibrillator;
pacemaker implantation;
transaortic septal myectomy;
excision of part of the interventricular septum;
transcatheter septal alcohol ablation.

Cardiomyopathy - treatment with folk remedies

On the recommendation of the treating cardiologist, you can supplement the main course with herbal remedies. Traditional treatment for left ventricular hypertrophy involves the use of viburnum berries without heat treatment, 100 g per day. It is useful to consume flax seeds, which have a positive effect on heart cells. Recommend:

take a spoonful of seeds;
add boiling water - liter;
hold in a water bath for 50 minutes;
filter;
drink per day - dose 100 g.

Oat infusion for regulating the functioning of the heart muscles has good reviews in the treatment of HCM. According to the healers' prescription, it is required:

oats – 50 grams;
water – 2 glasses;
heat to 50 degrees;
add 100 g of kefir;
pour in radish juice - half a glass;
stir, stand for 2 hours, strain;
put 0.5 tbsp. honey;
dosage – 100 g, three times a day before meals;
course – 2 weeks.

sovets.net

Definition. Left ventricular myocardium (LVMH) is an excess of the mass of the left ventricle relative to its proper value due to thickening (proliferation) of the myocardium (heart muscle).

Methods for diagnosing LVMH. Currently, 3 instrumental methods are used to diagnose LVMH:

— Standard ECG. When verifying LVMH, a conventional ECG is generally characterized by low sensitivity - no more than 30%. In other words, of the total number of patients who objectively have LVMH, an ECG allows it to be diagnosed in only a third. However, the more pronounced the hypertrophy, the higher the likelihood of recognizing it using a regular ECG. Severe hypertrophy almost always has ECG markers. Thus, if the ECG correctly diagnoses LVMH, this most likely indicates its severe degree. Unfortunately, in our medicine, a conventional ECG is given too much importance in diagnosing LVMH. Often, using low-specific ECG criteria for LVMH, doctors speak affirmatively about the phenomenon of hypertrophy where it actually does not exist. You should not expect more from a standard ECG than it actually shows.

— Ultrasound of the heart. It is the “gold standard” in the diagnosis of LVMH, as it allows real-time visualization of the walls of the heart and the necessary calculations. To assess myocardial hypertrophy, it is customary to calculate relative values that reflect myocardial mass. However, for simplicity, it is permissible to know the value of only two parameters: the thickness of the anterior (interventricular septum) and posterior wall of the left ventricle, which makes it possible to diagnose hypertrophy and its degree.

— Magnetic resonance imaging (MRI)). An expensive method of layer-by-layer scanning of the “zone of interest”. To assess LVMH, it is used only if for some reason ultrasound of the heart is not feasible: for example, in a patient with obesity and pulmonary emphysema, the heart will be covered on all sides by lung tissue, which will make its ultrasound visualization impossible (extremely rare, but this does occur).

The thickness of the IVS and the left ventricular myocardium is directly related to left ventricular myocardial hypertrophy (the clinical significance of the EDR during hypertrophy will be discussed). If the normal value of even one of the two presented parameters is exceeded, we can speak of “hypertrophy”.

Causes and pathogenesis of LVMH. Clinical conditions that can lead to LVMH (in order of decreasing frequency):

1. Diseases leading to increased afterload on the heart:

— Arterial hypertension (hypertension, secondary hypertension)

— Heart defect (congenital or acquired) - aortic stenosis.

Afterload is understood as a set of physical and anatomical parameters of the cardiovascular body that create an obstacle to the passage of blood through the arteries. Afterload is determined mainly by the tone of the peripheral arteries. A certain basic value of arterial tone is the norm and one of the obligate manifestations of homeostasis, maintaining the level of blood pressure, according to the current needs of the body. An excessive increase in arterial tone will mark an increase in afterload, which is clinically manifested by an increase in blood pressure. So, with spasm of the peripheral arteries, the load on the left ventricle increases: it needs to contract more strongly in order to “push” blood through the narrowed arteries. This is one of the main links in pathogenesis in the formation of a “hypertensive” heart.

The second common cause that leads to an increase in afterload on the left ventricle, and therefore creates an obstacle to arterial blood flow, is aortic stenosis. With aortic stenosis, the aortic valve is affected: it shrinks, calcifies, and becomes deformed. As a result, the aortic opening becomes so small that the left ventricle must contract significantly more to ensure that an adequate volume of blood passes through the critical bottleneck. Currently, the main cause of aortic stenosis is senile (senile) valve damage in the elderly.

Microscopic changes during myocardial hypertrophy include thickening of the cardiac fibers and some proliferation of connective tissue. At first, this is compensatory in nature, but with a long-term increased afterload (for example, with long-term untreated hypertension), hypertrophied fibers undergo dystrophic changes, the architectonics of the myocardial syncytium is disrupted, and sclerotic processes in the myocardium are predominant. As a result, hypertrophy turns from a compensation phenomenon into a mechanism for the manifestation of heart failure - the heart muscle cannot work under tension for an indefinitely long time without consequences.

2. Congenital cause of LVMH: hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy is a genetically determined disease, which is characterized by the appearance of unmotivated LVMH. The manifestation of hypertrophy occurs after birth: as a rule, in childhood or adolescence, less often in adults, but in any case no later than 35-40 years. Thus, in hypertrophic cardiomyopathy, LVMH occurs against a background of complete well-being. This disease is not very rare: according to statistics, 1 person out of 500 suffers from it. In my clinical practice, I annually see 2-3 patients with hypertrophic cardiomyopathy.

Unlike a hypertensive heart, with hypertrophic cardiomyopathy, LVMH can be very pronounced (severe) and often asymmetrical (more on this in more detail). Only with hypertrophic cardiomyopathy does the thickness of the wall of the left ventricle sometimes reach “exorbitant” values of 2.5-3 cm or more. Microscopically, the architecture of the cardiac fibers is grossly disrupted.

3. LVMH as a manifestation of systemic pathological processes.

— Obesity. Excess body weight is not solely a cosmetic problem. This is a deep pathophysiological process affecting all organs and systems, during which biochemical processes, psychodynamics of thinking, human selfhood, etc. change. With obesity, adipose tissue is deposited in excess not only under the skin, but also in almost all organs. The heart is forced to provide blood to “the body with all its excess mass.” Such an increased load cannot but affect cardiac functionality - it certainly increases: the heart contracts more often and stronger. Thus, in obesity, LVMH can develop in the absence of persistent arterial hypertension.

In obesity, the myocardium thickens not only due to the proliferation of cardiac fibers and connective tissue, but also due to the deposition of excess fat.

— Amyloidosis(primary or secondary) - a pathology in which a special amyloid protein is deposited in the internal organs, leading to the development of diffuse sclerosis and organ failure. Despite the possibility of developing LVMH due to amyloidosis, it rarely comes to the fore in the clinical picture of the disease: other organs (for example, kidneys) are more significantly affected, which will determine the specific picture of the disease.

4. Relatively natural causes of LVH.

— Elderly age. Senile age is characterized by slow but steadily progressive degradation (dystrophy) of all organs and systems. The specific gravity of water and parenchymal components in organs decreases; on the contrary, sclerotic processes intensify. The heart of an old man is no exception: the muscle fibers become thinner and loosen, at the same time, the connective tissue develops powerfully, due to which LVMH primarily occurs in old age. What is important to know is that senile LVMH, in the absence of other causes, never reaches significant values. It does not exceed the degree of “insignificant” and is more often only an age-related phenomenon, without any particular clinical significance.

— The heart of an athlete. We are talking about people who have been involved in professional sports for a long time. LVMH in such subjects can be called purely compensatory (working), as well as the concomitant hypertrophy of skeletal muscles. After the end of a sports career, LVMH undergoes complete or partial regression.

The following diseases (conditions) lead to concentric LVMH:

S-hypertrophy has no particular clinical significance, being more often a marker of an “age-related” heart. Occasionally, this type of hypertrophy occurs in middle-aged people.

Clinical significance of LVMH. Diseases leading to the development of LVMH can be asymptomatic for a long time (years, decades) or have nonspecific manifestations: for example, headache due to arterial hypertension. The earliest symptom of LVH (which, by the way, can appear after years of hypertrophy) is dyspnea with usual physical activity: walking, climbing stairs. Mechanism of shortness of breath: РґРёР°СЃС‚РѕР»РёС‡РµСЃРєР°СЏ SЃРµСЂРґРµС‡РЅР°СЏ РЅРµРґРѕСЃС‚Р°С‚РѕС‡РШРѕСЃС‚СЊ. It is known that blood filling of the heart occurs during diastole (relaxation): blood moves along a concentration gradient from the atria to the ventricles. With hypertrophy, the left ventricle becomes thicker, stiffer, denser - this leads to the fact that the process of relaxation and stretching of the heart becomes more difficult and incomplete; Accordingly, the blood supply to such a ventricle is disrupted (decreased). Clinically, this phenomenon manifests itself as shortness of breath. Symptoms of diastolic heart failure in the form of shortness of breath and weakness may be the only manifestation of LVMH for many years. However, in the absence of adequate treatment of the underlying disease, the symptoms will gradually increase, leading to a progressive decrease in exercise tolerance. The final outcome of advanced diastolic heart failure will be the development of systolic heart failure, the treatment of which is even more difficult. So, LVMH is a direct path to heart failure, which means a high risk of early cardiac death.

The next common complication of LVMH is development of paroxysmal atrial fibrillation (atrial fibrillation). Impaired relaxation (diastole) of the hypertrophied left ventricle inevitably entails an increase in blood pressure in it; this in turn causes the left atrium to contract more strongly in order to “push” the required volume of blood into the “container” with increased pressure. However, the left atrium is a thin-walled cardiac chamber that cannot operate in super mode for a long time; As a result, the left atrium dilates (widens) to accommodate the excess blood. Dilatation of the left atrium is one of the most important risk factors for the development of atrial fibrillation. As a rule, damage to the left atrium for a long time is manifested only by atrial extrasystole; subsequently, when the atrium “dilates sufficiently” to “maintain” fibrillation, atrial fibrillation occurs: first paroxysmal, then constant. The risks that atrial fibrillation brings into a patient’s life are described in detail in a separate chapter.

Obstructive syncope. A rare variant of the course of LVMH. It is almost always a complication of the asymmetric variant of hypertrophic cardiomyopathy, when the thickness of the interventricular septum is so great that there is a threat of transient obstruction (blocking) of blood flow in the area of the outflow tract of the left ventricle. Paroxysmal obstruction (cessation) of blood flow in this “critical place” will inevitably lead to fainting. As a rule, the risk of developing obstruction occurs when the thickness of the interventricular septum exceeds 2 cm.

Ventricular extrasystole- another possible satellite for LVMH. It is known that any micro- and macroscopic changes in the heart muscle can theoretically be complicated by extrasystole. Hypertrophied myocardium is an ideal arrhythmogenic substrate. The clinical course of ventricular extrasystole against the background of LVMH is variable: more often, its role is limited to a “cosmetic arrhythmic defect”. However, if the disease leading to LVMH is not treated (ignored), and the regimen for limiting intense physical activity is not followed, life-threatening ventricular arrhythmias triggered by extrasystole may develop.

Sudden cardiac death. The most severe complication of LVMH. Most often, LVMH due to hypertrophic cardiomyopathy leads to this outcome. There are two reasons. Firstly, in this disease, LVMH can be particularly massive, which makes the myocardium extremely arrhythmogenic. Secondly, hypertrophic cardiomyopathy very often has an asymptomatic course, which does not allow patients to take preventive measures in the form of limiting intense physical activity. Sudden cardiac death in other nosologies complicated by LVMH is generally a rare phenomenon, if only because the manifestation of these diseases begins with symptoms of heart failure, which in itself forces the patient to see a doctor, which means there is a real opportunity to take the disease under control.

Possibility of regression of LVMH. The likelihood of a decrease in the mass (thickness) of the left ventricular myocardium during treatment depends on the cause of hypertrophy and its degree. A classic example is the athletic heart, the walls of which can decrease to normal thickness after the end of a sports career.

LVMH due to arterial hypertension or aortic stenosis can successfully regress with timely, complete and long-term control of these diseases. However, it is considered this way: only mild hypertrophy undergoes absolute regression; when treating moderate hypertrophy, there is a chance of reducing it to mild; and heavy can “become medium”. In other words, the more advanced the process, the less likely it is to return everything completely to its original state. However, any degree of regression of LVMH automatically means correctness in the treatment of the underlying disease, which in itself reduces the risks that hypertrophy introduces into the subject’s life.

With hypertrophic cardiomyopathy, any attempts at drug correction of the process are pointless. There are surgical approaches for the treatment of massive hypertrophy of the interventricular septum, which is complicated by obstruction of the left ventricular outflow tract.

The likelihood of regression of LVMH due to obesity, in older people, and with amyloidosis is practically absent.

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Version: MedElement Disease Directory

Other hypertrophic cardiomyopathy (I42.2)

general information

Short description

Non-obstructive hypertrophic cardiomyopathy

Isolated myocardial hypertrophy of unknown nature was first described in the second half of the 19th century by French pathologists N. Lionville (1869) and L. Hallopeau (1869). They noted a narrowing of the outflow tract of the left ventricle due to thickening of the interventricular septum and gave this disease the name “left-sided muscular conus stenosus.”

Hypertrophic cardiomyopathy- a myocardial disease of unknown etiology, inherited in an autosomal dominant manner, characterized by hypertrophy of the myocardium of the left and (or) occasionally right ventricle, more often, but not necessarily, asymmetric, as well as severe disturbances in the diastolic filling of the left ventricle in the absence of dilatation of its cavity and the reasons causing hypertrophy hearts.

Classification

Classification of cardiomyopathies

By localization

1. LV hypertrophy

a) Asymmetrical:

Hypertrophy of the basal parts of the IVS

Total hypertrophy of the IVS

Total hypertrophy of the IVS and LV free wall

Hypertrophy of the cardiac apex with possible extension to the LV free wall and septum

b) Symmetrical (concentric)

2. RV hypertrophy

I I. According to the hemodynamic form

1. Non-obstructive

2. Obstructive

III. By pressure gradient(with obstructive form)

Stage 1 - pressure gradient less than 25 mmHg

Stage 2 - less than 36 mmHg

Stage 3 - less than 44 mmHg

Stage 4 - from 45 mmHg

IV . According to clinical manifestations

Asymptomatic course

Syncope

Rhythm disorders

V. By clinical stage

1. Compensation

2. Subcompensation

Etiology and pathogenesis

HCM is a hereditary disease that is transmitted as an autosomal dominant trait. A genetic defect occurs when a mutation occurs in one of 10 genes, each of which encodes components of the cardiac sarcomere protein and determines the development of myocardial hypertrophy. Currently, about 200 mutations responsible for the development of the disease have been identified.

There are several pathogenetic mechanisms for the development of the disease:

- Hypertrophy of the interventricular septum. As a result of a genetic defect in the myocardial sarcomere, disproportionate hypertrophy of the interventricular septum can develop, which in some cases occurs even during embryonic morphogenesis. At the histological level, changes in the myocardium are characterized by the development of metabolic disorders in the cardiomyocyte and a significant increase in the number of nucleoli in the cell, which leads to the disintegration of muscle fibers and the development of connective tissue in the myocardium (English phenomenon “disarray” - the phenomenon of “disorder”). Disorganization of cardiac muscle cells and replacement of the myocardium with connective tissue lead to a decrease in the pumping function of the heart and serve as the primary arrhythmogenic substrate, predisposing to the occurrence of life-threatening tachyarrhythmias.
- Obstruction of the left ventricular outflow tract. Of great importance in HCM is LVOT obstruction, which occurs as a result of disproportionate hypertrophy of the interventricular septum, which promotes contact of the anterior leaflet of the mitral valve with the interventricular septum and a sharp increase in the pressure gradient in the LVOT during systole.
- Impaired relaxation of the left ventricular myocardium. The long-term existence of obstruction and hypertrophy of the interventricular septum leads to a deterioration in active muscle relaxation, as well as an increase in the rigidity of the LV walls, which causes the development of LV diastolic dysfunction, and in the terminal phase of the disease - systolic dysfunction.
- Myocardial ischemia. An important part of the pathogenesis of HCM is myocardial ischemia, which is associated with the development of left ventricular hypertrophy and diastolic dysfunction, which leads to hypoperfusion and increased myocardial fiber dissection. As a result, thinning of the walls of the left ventricle occurs, its remodeling and the development of systolic dysfunction.

Epidemiology

Hypertrophic cardiomyopathy occurs with an incidence of 1:1000-1:500. It is generally accepted that it is most common among residents of Asia and the Pacific coast, especially in Japan. Men get sick more often than women. It is more common in young people, being a common cause of sudden cardiac death in them. About half of all cases of the disease are familial. The annual mortality rate from HCM is 1-6%.

Risk factors and groups

Risk factors for sudden death in hypertrophic cardiomyopathy:

Manifestation of the disease at a young age (up to 16 years),
- a family history of episodes of sudden death,
- frequent syncope,
- short episodes of ventricular tachycardia detected during 24-hour ECG monitoring,
- pathological changes in blood pressure during exercise.

Clinical picture

Symptoms, course

HCM can manifest at any age. The clinical presentation is usually variable, and patients may remain stable for a long period of time.

Classic triad of symptoms for hypertrophic cardiomyopathy includes angina pectoris, shortness of breath on exertion and syncope. Pain in the chest is observed in 75% of patients with hypertrophic cardiomyopathy, classic angina pectoris in 25%.

Dyspnea and often accompanying chest pain, dizziness, syncope and presyncope usually occur with preserved LV systolic function. The listed symptoms are associated with the occurrence of diastolic myocardial dysfunction and other pathophysiological mechanisms (myocardial ischemia, LVOT obstruction and concomitant mitral regurgitation, AF).

Chest pain in the absence of atherosclerotic lesions of the coronary vessels, it can be either typical for angina or atypical.

Syncope and dizziness are typical, first of all, for patients with an obstructive form of HCM due to hemodynamic obstruction (reduction of the LVOT lumen). In most cases, they appear suddenly against the background of complete health during a period of physical or emotional stress, however, they can also occur at rest. Most often, fainting is observed in young patients; in many of them, during daily ECG monitoring, episodes of ventricular tachycardia and conduction disturbances are recorded.

A significant number of patients (5-28%) experience atrial fibrillation, which increases the risk of thromboembolic complications.

An objective examination of patients with a non-obstructive form of hypertrophic cardiomyopathy may not show any deviations from the norm, but sometimes an increase in the duration of the apex beat and IV heart sound are detected.

Diagnostics

ECG in 12 leads.

Various ECG changes are recorded in 92-97% of patients; they serve as the earliest manifestation of HCM and may precede the development of myocardial hypertrophy detected by echocardiography. There are no strictly specific ECG signs of HCM, as well as clinical ones.
The most common changes are the ST segment, T wave inversion, signs of more or less pronounced left ventricular hypertrophy, deep Q waves and signs of hypertrophy and overload of the left atrium. Less common are blockade of the anterosuperior branch of the left bundle branch and signs of hypertrophy of the right atrium, and in isolated cases of the right ventricle. Complete bundle branch block is not typical. Common ECG changes in HCM are negative T waves, in some cases in combination with ST segment depression, which are recorded in 61-81% of patients. Giant, more than 10 mm deep, negative T waves in the chest leads are very characteristic of the apical form of this disease, in which they have important diagnostic value. Changes in the final part of the ventricular complex in HCM are caused by myocardial ischemia or small-focal cardiosclerosis. The detection of deep Q waves and negative T waves, especially with complaints of anginal pain, is a common cause of misdiagnosis of IHD and necessitates the differential diagnosis of HCM with this disease.

Holter ECG monitoring. Holter ECG monitoring to diagnose rhythm and conduction disorders is indicated for patients at high risk of sudden death, primarily with syncope, a history of sudden death in the family, as well as clinical and ECG signs of myocardial ischemia. It is also advisable to use it to monitor the effectiveness of antiarrhythmic therapy.

Phonocardiography. A very characteristic, but nonspecific, pathological increase in the third and especially fourth heart sounds. An important sign of subaortic obstruction is the so-called late, not associated with the first sound, systolic murmur of a rhomboid or ribbon shape with an epicenter at the apex or in the third-fourth intercostal space at the left edge sternum. It is carried out in the axillary region and, less commonly, at the base of the heart and vessels of the neck. Distinctive features of noise that allow one to suspect obstructive HCM are specific changes in its amplitude and duration during physiological and pharmacological tests aimed at increasing or decreasing the degree of obstruction and associated mitral insufficiency. This type of noise dynamics has not only diagnostic significance, but is also a valuable criterion for the differential diagnosis of HCM with primary lesions of the mitral and aortic valves. The noise may be preceded by an additional tone that is formed when the mitral cusp comes into contact with the interventricular septum. In some patients, a short, low-amplitude inflow murmur following the third tone is recorded in diastole, that is, relative mitral or, occasionally, tricuspid stenosis. In the latter case, the noise intensifies on inspiration. If the obstruction to blood flow is significant, a paradoxical splitting of the second tone is determined due to the prolongation of the ejection period of the left ventricle in proportion to the magnitude of the systolic pressure gradient.

X-ray examination of the chest. Data from X-ray examination of the heart are not very informative. Even with significant myocardial hypertrophy, significant changes in the heart shadow may be absent, since the volume of the left ventricular cavity is not changed or reduced. In some patients, there is a slight increase in the arcs of the left ventricle and left atrium and rounding of the apex of the heart, as well as signs of moderate venous pulmonary hypertension. The aorta is usually reduced.

Doppler echocardiography
None of the echocardiographic signs of HCM, despite its high sensitivity, is pathognomonic.

Main echocardiographic signs :
- Asymmetric myocardial hypertrophy of the left ventricle A. The generally accepted criterion for HCM is a thickness of the interventricular septum of more than 15 mm with normal or increased thickness of the posterior wall of the LV. Considering that the disease is genetically determined, the degree of hypertrophy may vary. However, the presence of symmetrical hypertrophy does not exclude the diagnosis of HCM.

- Obstruction of the outflow tract of the left ventricle. Hemodynamic systolic pressure gradient in the LVOT is determined using Doppler scanning. A gradient of more than 30 mmHg is considered diagnostically significant. (flow velocity in the LVOT is 2.7 m/s). A test with physical activity is performed to determine the degree of gradient in the LVOT. The dobutamine test is not used due to the high risk of developing life-threatening arrhythmias.
- Anterior systolic movement of the anterior mitral valve leaflet. Left atrial dilatation, mitral regurgitation, and, in the terminal stage, LV dilatation are also often detected.

Stress EchoCG used to detect coronary heart disease concomitant with HCM, which has important prognostic and therapeutic significance.

Radionuclide ventriculography As the most reproducible method for assessing the systolic and diastolic function of not only the left but also the right ventricle, it is used mainly to monitor patients with HCM over time and to assess the effectiveness of treatment measures.

Magnetic resonance imaging with It is the most accurate method for assessing cardiac morphology, which plays a key role in the diagnosis of HCM. Thus, magnetic resonance imaging allows one to obtain additional information, compared to echocardiography, about the distribution of hypertrophy in 20-31% of patients with HCM (F. Sardinelli et al., 1993; J. Posma et al., 1996) and provides measurements of the thickness of 97% of segments left ventricle compared to 67% when using echocardiography (G. Pons-Llado et al., 1997). Thus, magnetic resonance imaging can serve as a kind of “gold standard” for assessing the prevalence and severity of myocardial hypertrophy in patients with HCM.

Positron emission tomography presents a unique opportunity for non-invasive assessment of regional myocardial perfusion and metabolism. Preliminary results of its use in HCM showed a decrease in the coronary expansion reserve not only in hypertrophied, but also in unchanged thickness segments of the left ventricle, which is especially pronounced in patients with anginal pain. Impaired perfusion is often accompanied by subendocardial ischemia

When measuring pressure in the cavities of the heart The most important diagnostic and therapeutic value is the detection of the systolic pressure gradient between the body and the outflow tract of the left ventricle at rest or during provocative tests. This sign is characteristic of obstructive HCM and is not observed in the non-obstructive form of the disease, which does not allow excluding HCM in its absence. When recording the pressure gradient in the cavity of the left ventricle in relation to its outflow tract, it is necessary to make sure that it is caused by subaortic obstruction of blood expulsion, and is not a consequence of tight grasping of the end of the catheter by the walls of the ventricle during the so-called “elimination” or “obliteration” of its cavity. Along with the subaortic gradient, an important sign of obstruction of blood expulsion from the left ventricle is a change in the shape of the pressure curve in the aorta. As in the sphygmogram, it takes on the shape of a “peak and dome.” In a significant proportion of patients with HCM, regardless of the presence or absence of a subaortic gradient, an increase in end-diastolic pressure in the left ventricle and pressure in the paths of its inflow is determined - in the left atrium, pulmonary veins , "pulmonary capillaries" and pulmonary artery. In this case, pulmonary hypertension is passive, venous. The increase in end-diastolic pressure in the hypertrophied left ventricle is due to a violation of its diastolic compliance, characteristic of HCM. Sometimes, in the terminal stage of the disease, it is aggravated as a result of the addition of systolic myocardial dysfunction.

Coronary angiography. It is performed for HCM and constant chest pain (frequent attacks of angina):

In persons over 40 years of age;
in persons with risk factors for coronary artery disease;
in persons with an established diagnosis of coronary artery disease before invasive intervention (for example, septal myectomy or alcohol septal ablation).

Endomyocardial biopsy left or right ventricle testing is recommended in cases where, after clinical and instrumental examination, doubts remain regarding the diagnosis. When characteristic pathohistological signs of the disease are identified, a conclusion is made about the correspondence of morphological changes in the myocardium to the clinical diagnosis of HCM. On the other hand, the detection of structural changes specific to any other myocardial lesion (for example, amyloidosis) allows us to exclude HCM.

With the availability of Doppler echocardiography and magnetic resonance imaging, EMB is now practically not used for diagnosing HCM.

Laboratory diagnostics

In order to exclude other most common cardiac diseases, it is necessary to conduct a biochemical blood test (lipid spectrum, biomarkers of myocardial necrosis, electrolyte composition of blood, serum glucose), assessment of the functional state of the kidneys, liver and general clinical tests of blood and urine.

Differential diagnosis

Differential diagnosis is carried out with a number of diseases accompanied by the development of left ventricular hypertrophy, primarily “athlete's heart”, acquired and congenital defects, dilated cardiomyopathy, and with a tendency to increase blood pressure - essential arterial hypertension. Differential diagnosis of heart defects accompanied by systolic murmur becomes especially important in cases of obstructive HCM. In patients with focal and ischemic changes on the ECG and anginal pain, the primary task is differential diagnosis with ischemic heart disease. If signs of congestive heart failure predominate in the clinical picture in combination with a relatively small increase in heart size, HCM should be differentiated from atrial myxoma, chronic pulmonary heart disease and diseases occurring with restriction syndrome - constrictive pericarditis, amyloidosis, hemochromatosis and sarcoidosis of the heart and restrictive cardiomyopathy.

"The heart of an athlete." Differential diagnosis of non-obstructive HCM, especially with relatively mild left ventricular hypertrophy (wall thickness 13-15 mm), from the “athlete's heart” is a difficult task, quite often encountered in sports medicine. The importance of its decision is due to the fact that HCM is the leading cause of death in young professional athletes, and therefore such a diagnosis serves as a basis for their disqualification. Probable HCM in these controversial cases is indicated by the presence of other changes on the ECG, in addition to signs of left ventricular hypertrophy. Doppler echocardiography in favor of HCM is indicated by an unusual distribution of myocardial hypertrophy, a decrease in the end-diastolic diameter of the left ventricle of less than 45 mm, an increase in the size of the left atrium and other signs of impaired diastolic filling of the left ventricle.

Cardiac ischemia. Most often, HCM has to be differentiated from chronic and less often acute forms of coronary artery disease. In both cases, anginal pain in the heart area, shortness of breath, cardiac arrhythmias, concomitant arterial hypertension, additional sounds in diastole, small and large focal changes and signs of ischemia on the ECG may be observed. EchoCG is important for making a diagnosis, in which in some patients impairments of segmental contractility characteristic of ischemic heart disease, moderate dilation of the left ventricle and a decrease in its ejection fraction are determined. Left ventricular hypertrophy is very moderate and is often symmetrical. The impression of disproportionate thickening of the interventricular septum can be created by the presence of zones of akinesia due to post-infarction cardiosclerosis in the posterior wall of the left ventricle with compensatory hypertrophy of the septal myocardium. Moreover, in contrast to asymmetric hypertrophy of the interventricular septum as a form of HCM, septal hypertrophy is accompanied by hyperkinesia. In cases of noticeable dilatation of the left atrium due to concomitant mitral regurgitation in ischemic heart disease, dilatation of the left ventricle is invariably observed, which is unusual in patients with HCM. The diagnosis of HCM can be confirmed by detecting signs of a subaortic pressure gradient. In the absence of echocardiography data in favor of subaortic obstruction, differential diagnosis becomes significantly more difficult. The only reliable method for recognizing or excluding coronary artery disease in such cases is radiopaque coronary angiography. In middle-aged and older people, especially men, it is necessary to keep in mind the possibility of combining HCM with ischemic heart disease.

Essential arterial hypertension. For differential diagnosis, the greatest difficulty is presented by HCM, which occurs with an increase in blood pressure, which should be distinguished from isolated essential arterial hypertension, accompanied by left ventricular hypertrophy with disproportionate thickening of the interventricular septum. Essential arterial hypertension is evidenced by a significant and persistent increase in blood pressure, the presence of retinopathy, as well as an increase in the thickness of the intima and media of the carotid arteries, which is not typical for patients with HCM. Particular attention should be paid to identifying signs of subaortic obstruction. In the absence of a subaortic pressure gradient, probable HCM, in contrast to essential arterial hypertension, is indicated by the significant severity of asymmetric hypertrophy of the interventricular septum with an increase in its thickness by more than 2 times compared with the posterior wall of the left ventricle, as well as the detection of HCM in at least one of 5 adult blood relatives. On the contrary, if there are no signs of HCM in 5 or more family members of the patient, the probability of this disease does not exceed 3%.

Complications

The course of the disease may be complicated by the development of complications such as:

Sudden cardiac death (SCD)
- thromboembolism
- progression of chronic heart failure (CHF).

Treatment

TO general events include limiting significant physical activity and prohibiting sports that can worsen myocardial hypertrophy, increase the intraventricular pressure gradient and the risk of VS. To prevent infective endocarditis in situations associated with the development of bacteremia, with obstructive forms of HCM, antibiotic prophylaxis is recommended, similar to that in patients with heart defects.

The basis of drug therapy for HCM is drugs with a negative inotropic effect: β-blockers and calcium channel blockers (verapamil).

β-blockers became the first and remain to this day the most effective group of drugs used in the treatment of HCM. they should be prescribed to patients regardless of the severity of the intraventricular pressure gradient at rest. It is preferable to refrain from prescribing β-aope-noceptor blockers with intrinsic sympathomimetic activity (pin-dolol, oxprenolol). Propranolol is prescribed at a dose of 240-320 mg per day or more (maximum daily dose - 480 mg), metoprolol - at a dose of 200 mg per day or more. Cardioselective beta-adrenoreceptor blockers for hypertrophic cardiomyopathy have no advantages over non-selective ones, since in high doses selectivity is practically lost.

In case of contraindications to the use of beta-adrenergic blockers or incomplete resolution of symptoms, an alternative may be calcium channel blockers. Among calcium channel blockers, the drug of choice is verapamil (isoptin, finoptin). It provides a symptomatic effect in 65-80% of patients. When prescribing calcium channel blockers, maximum caution is necessary in the presence of severe hypertrophy and very high left ventricular filling pressure. It should be borne in mind that calcium channel blockers, including verapamil, may increase diastolic pressure and reduce cardiac output when used over a long period of time. Treatment with verapamil should begin with low doses - 20-40 mg 3 times a day, gradually increasing to a daily dose of 240-320 mg or more. Clinical improvement when taking verapamil is accompanied by an increase in exercise tolerance.

Antiarrhythmic drugs are used to treat arrhythmias, of which the most effective are disopyramide and amiodarone. Disopyramide (rhythmilen), a class IA antiarrhythmic, has a pronounced negative inotropic effect; in patients with HCM it can reduce the level of obstruction of the left ventricular outflow tract and has a positive effect on the structure of diastole. The initial dose is usually 400 mg/day with a gradual increase to 800 mg. In this case, it is necessary to monitor the duration of the Q-T interval using an ECG.
The only drug that has currently been shown to eliminate ventricular tachyarrhythmia, reduce the incidence of sudden death and improve the prognosis of the disease is amiodarone. Amiodarone is prescribed at a daily dose of 1200 mg for 5-7 days, then at a daily dose of 800 mg and 600 mg during the 2nd and 3rd weeks of treatment, followed by a transition to a maintenance daily dose of 200 mg.

When treating HCM with symptoms of heart failure, the following points should be considered:
diuretics are contraindicated, which, although they effectively reduce pulmonary congestion, can cause hypovolemia, which can increase outflow tract obstruction in patients! Excessive use of diuretics may cause a decrease in stroke volume and cardiac output.
Vasodilators (nitroglycerin, sodium nitroprusside) have limited use due to the possible risk of developing severe hypotension and reducing the size of the left ventricular cavity, which can worsen the patient’s condition.
Inotropic agents aimed at stimulating systolic output (cardiac glycosides and pressor amines), can have an unfavorable hemodynamic effect - they increase the obstruction of the outflow tract and do not reduce the increased end-diastolic pressure, and can cause the development of asystole. However, digoxin can be used in patients with diastolic dysfunction and atrial fibrillation to reduce heart rate and/or restore sinus rhythm.
The drugs of choice for the treatment of CHF may be ACE inhibitors due to their ability to block the renin-angiotensin system and reverse left ventricular hypertrophy.

In the absence of clinical effect from active drug therapy in symptomatic patients of functional class III-IV according to the classification of the New York Heart Association with severe asymmetric IVS hypertrophy and a subaortic pressure gradient at rest equal to 50 mm Hg. Art. and more, surgical treatment is indicated (link to treatment from I42.1)

Forecast

Non-obstructive forms of HCM have, in general, a more favorable course with less severe limitation of the functional state and longer periods of stabilization. The prognosis is most favorable with a long asymptomatic course of the disease and an uncomplicated family history, especially with the apical form of HCM. In some such cases, the disease may not affect life expectancy.

Most patients with HCM die suddenly, regardless of how long the disease has been present. A poor prognosis in children, most of whom are asymptomatic, is associated with a strong family history of sudden death. In adolescents and young and middle-aged people (from 15 to 56 years), the main factor aggravating the prognosis is susceptibility to fainting. In older patients, shortness of breath and pain in the heart during physical activity are unfavorable prognostic factors.

There are 5 main options for the course of the disease and outcomes:
- stable, benign course;
- sudden death;
- progressive course - increased shortness of breath, weakness, fatigue, pain (atypical pain, angina), the appearance of syncope, disorders of LV systolic dysfunction;
- “final stage” - further progression of congestive heart failure associated with LV remodeling and systolic dysfunction;
- development of atrial fibrillation and associated complications, in particular thromboembolic ones.

Hospitalization

Indications for hospitalization

Clinically significant worsening of CHF symptoms.
- Heart rhythm disturbances: new, hemodynamically unstable, life-threatening.
- Circulatory arrest (asystole or ventricular fibrillation).
- Brain symptoms (syncope, presyncope).
- Persistent anginal pain.

Prevention

Prevention of the disease lies in identifying it in the early stages, which allows early treatment of the disease to begin and prevent the development of severe myocardial hypertrophy. EchoCG must be performed on the “blood” (genetic) relatives of the patient. All other persons are indicated for a detailed examination in the presence of manifestations similar to the disease: fainting, angina, etc. Screening (for everyone) ECG and EchoCG during the annual medical examination are also useful.

Information

Treatment of heart failure. Recommendations of the working group on heart failure of the European Society of Cardiology. Rus. honey. magazine Application. 1999.
Amosova E.N. Cardiomyopathies. Kyiv: "Book Plus", 1999; 421 p.
Kushakovsky M.S. Chronic congestive heart failure. Idiopathic myocardiopathies. St. Petersburg: "Foliant", 1998; 320 pp.
Moiseev V.S., Sumarokov A.V., Styazhkin V.Yu. Cardiomyopathies. M.: Medicine 1993; 176 p.
Guide to outpatient cardiology, edited by Belenkov Yu.N., Oganova R.G., publishing house GEOTAR-Media, - 2007.-400 p.
Polyakov V.P., Nikolaevsky V.N., Pichko G.A. Non-coronary and infectious diseases of the heart (modern aspects of the clinic, diagnosis, treatment): Monograph.-Samara, 2010,-355p.
Internal diseases according to Tinsley R. Harrison / Ed. E. Fauci, Y. Braunwald, K. Isselbacher, J. Wilson, J. Martin, D. Kasper, S. Hauser and D. Longo: In 7t. - M.: Praktika-McGraw_Hill, 2005.
Clinical recommendations. Cardiology / Ed. Yu.N. Belenkova, R.G. Oganova. - M.: GEOTAR-Media, 2007.
B. Griffin, E. Topol "Cardiology" Moscow, 2008
Kovalenko V. N., Nesukai E. G. Non-coronary heart diseases: a practical guide / Ed. V. N. Kovalenko. K.: "Morion", 2001. - 480 p.

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Content

Thickening of the wall of the myocardium (the muscular lining of the heart) is a pathological condition. In medical practice, there are various types of cardiomyopathy. Morphological changes in the main organ of the circulatory system lead to a decrease in the contractility of the heart, and insufficient blood supply occurs.

What is hypertrophic cardiomyopathy

A disease characterized by thickening (hypertrophy) of the wall of the left (rarely right) ventricle of the heart is called hypertrophic cardiomyopathy (HCM). The muscle fibers of the myocardium are located chaotically - this is a characteristic feature of the disease. In most cases, asymmetric thickening is observed, and hypertrophy of the interventricular septum develops.

The pathology is characterized by a decrease in ventricular volume and impaired pumping function. The heart has to beat frequently to deliver enough blood to the organs. The consequence of these changes is heart rhythm disturbances and the appearance of heart failure. 30–50 years is the average age of patients diagnosed with hypertrophic cardiomyopathy. The disease is more common in men. The pathological condition is recorded in 0.2–1.1% of the population.

Causes

HCM is a hereditary disease. Occurs as a result of gene mutation. The type of transmission of altered hereditary structures is autosomal dominant. Pathology is not only congenital. In some cases, mutations occur under the influence of unfavorable environmental factors. The consequences of changing the genetic code are as follows:

the biological synthesis of myocardial contractile proteins is disrupted;
muscle fibers have an incorrect location and structure;
muscle tissue is partially replaced by connective tissue, myocardial fibrosis develops;
altered cardiomyocytes (muscular membrane cells) work uncoordinated, with increased load;
muscle fibers thicken, myocardial hypertrophy occurs.

Thickening of the muscle membrane (compensatory hypertrophy) is caused by one of two pathological processes:

Violation of diastolic myocardial function. During the period of relaxation of the heart (diastole), the ventricle is not filled with blood enough due to poor distensibility of the myocardium. This leads to an increase in diastolic pressure.
Obstruction (impaired patency) of the left ventricular outflow tract. Myocardial hypertrophy of the interventricular septum occurs. Blood flow is hampered due to impaired mobility of the mitral valve leaflet. At the moment of blood ejection, a difference in systolic pressure occurs between the cavity of the left ventricle and the initial part of the aorta. For this reason, some of the blood is retained in the heart. As a result, left ventricular end-diastolic pressure increases. Myocardial hypertrophy, dilatation (expansion) of the left atrium are consequences of compensatory hyperfunction.

Classification

The criteria underlying the classification of the disease are different. The following types of illness are distinguished:

Criterion		Common diagnoses: asymmetric hypertrophy of the interventricular septum; hypertrophic obstructive cardiomyopathy; asymmetric hypertrophy of the apex of the heart (apical)
Localization	Right ventricular or left ventricular hypertrophy
Features of thickening formation	Asymmetrical, concentric (or symmetrical)
Changed structures	Hypertrophy of the interventricular septum, apex of the heart, anterolateral wall, posterior wall
The presence of a gradient (difference) of systolic pressure in the left ventricle	Obstructive, non-obstructive
Degree of myocardial thickening	Moderate – 15-20 mm, average – 21-25 mm, pronounced – more than 25 mm

Taking into account the prevailing complaints of patients, nine forms of pathology are distinguished. While there are general symptoms, each variant of HCM has specific symptoms. The clinical forms are as follows:

The clinical and physiological classification identifies four stages of disease development. The main criterion is the difference in systolic pressure in the left ventricular outflow tract (LVOT) and in the aorta:

The first stage is a pressure indicator in the LVOT no more than 25 mm Hg. Art. The patient has no complaints about the deterioration of his condition.
The second stage is a pressure gradient of about 36 mmHg. Art. The condition worsens with physical activity.
The third stage – the pressure difference is up to 44 mm Hg. Art. Shortness of breath is observed and angina develops.
The fourth stage is a systolic pressure gradient in the LVOT of more than 88 mmHg. Art. Blood circulation is impaired and sudden death is possible.

Symptoms of hypertrophic cardiomyopathy

The disease may not manifest itself for a long time. Hypertrophic cardiomyopathy is the main cause of death among young athletes who were unaware of the presence of a hereditary disease. 30% of patients with GTCS have no complaints and do not experience a deterioration in their general condition. The symptoms of the pathology are as follows:

fainting, dizziness, shortness of breath, cardialgia, angina pectoris and other conditions associated with low blood output syndrome;
left ventricular heart failure;
heart rhythm disturbances (extrasystoles, paroxysms, arrhythmias);
sudden death (in the absence of symptoms);
the occurrence of complications - infective endocarditis, thromboembolism.

Diagnostics

The first signs of the disease appear in childhood, but in most cases it is diagnosed in adolescence or in patients 30–40 years old. Based on a physical examination (assessment of external condition), the doctor makes a primary diagnosis. The examination helps to identify the expansion of the borders of the heart, listen to the characteristic systolic murmur, if there is an obstructive form of the disease, the emphasis of the second tone may be on the pulmonary artery. When examining the jugular veins for hypertrophy, poor contractility of the right ventricle is indicated by a well-defined A wave.

Additional diagnostic methods include:

Electrocardiography. In the presence of pathology, the ECG is never normal. The study allows us to determine the enlargement of the heart chambers, disturbances in conduction and contraction frequency.
Chest X-ray. Helps identify changes in the size of the atria and ventricles.
Echocardiography. The main method for identifying the location of thickening of the heart wall, the degree of blood flow obstruction, and diastolic dysfunction.
Electrocardiogram monitoring throughout the day, with the use of physical activity. The method is important for preventing sudden death, prognosis of the disease, and identifying heart rhythm disturbances.
Radiological methods. Ventriculography (examination of the heart with the introduction of a contrast agent) and magnetic resonance imaging (MRI) are performed. They are used in complex cases to identify and accurately assess pathological changes.
Genetic diagnostics. The most important method for assessing the prognosis of the disease. Genotype analysis is carried out on the patient and his family members.

Treatment of hypertrophic cardiomyopathy

If a patient has symptoms of hypertrophic cardiomyopathy, a wide range of medications are used. If drug therapy is ineffective in the case of an obstructive form of pathology, surgical and alternative interventional methods of correction are used. A special treatment regimen is prescribed for patients with a high risk of sudden death or the last stage of the disease. The goals of therapy are:

reducing the severity of symptoms and clinical manifestations of pathology;
increasing the patient’s “quality of life”, improving functional ability;
ensuring a positive prognosis of the disease;
prevention of cases of sudden death and progression of the disease.

Drug therapy

In patients with symptoms of HCM, restriction of physical activity is recommended. This rule should be strictly followed by patients with an obstructive form of the disease. Loads provoke the development of arrhythmias, fainting, and an increase in the LVOT pressure gradient. To alleviate the condition with moderate symptoms of HCM, drugs of different pharmacological groups are prescribed:

Beta blockers (Propranolol, Metoprolol, Atenolol) or calcium channel blockers (Verapamil). They reduce heart rate, lengthen diastole (relaxation phase), improve filling of the ventricles with blood, and reduce diastolic pressure.
Calcium antagonists (Finoptin, Amiodarone, Cardil). Medicines reduce the amount of calcium in the coronary arteries, improve relaxation of structures (diastole), and stimulate myocardial contractility.
Anticoagulants (Phenindione, Heparin, Bivalirudin). Medicines reduce the risk of thromboembolism.
Diuretics (Furosemide, Indapamed), ACE inhibitors (Captopril, Ramipril, Fosinopril). The drugs are recommended for patients with heart failure.
Antiarrhythmic drugs (Disopyramide, Amiodarone).

In case of HCM, the use of cardiac glycosides, nifedipine, and nitrites is contraindicated. These drugs contribute to the development of obstruction.

Surgery

Cardiac surgical treatment is advisable in the absence of effectiveness of taking pharmacological drugs. Surgery is indicated for patients in whom the pressure difference between the left ventricle (LV) and the aorta is more than 50 mm Hg. Art. at rest and during physical activity. To alleviate the patient's condition, the following surgical techniques are used:

Transaortic septal myectomy (TSM). It is recommended for patients who experience fainting, shortness of breath, or chest pain during physical activity. The essence of the operation is to remove part of the interventricular septum. This manipulation ensures good LV contractility and free movement of blood into the aorta.
Percutaneous alcohol ablation. The operation is prescribed for patients with contraindications to EMS, elderly patients who have inadequate blood pressure under stress conditions. Sclerosing substances (for example, alcohol solutions) are injected into the hypertrophied interventricular septum.
Dual-chamber pacing. The technique is used for patients with contraindications to surgery. The manipulation improves heart function and increases cardiac output.
Implantation of an artificial mitral valve. Prosthetics are recommended for patients in whom poor blood flow is not due to thickening of the interventricular septum, but as a result of inversion of the valve into the aortic lumen.
Installation of ICD (implantable cardioverter-defibrillator). Indications for such a procedure are a high risk of sudden death, previous cardiac arrest, and persistent ventricular tachycardia. To install an ICD, an incision is made in the subclavian region, the electrodes are inserted through a vein (there can be 1-3, depending on the pacemaker model) and installed under x-ray control inside the heart.
Heart transplantation. Prescribed to patients with the last stage of heart failure, in the absence of effect from drug treatment.

Surgical operations significantly improve the condition of patients and increase tolerance to physical activity. Surgical treatment does not protect against further development of pathological thickening of the myocardium and complications. In the postoperative period, the patient must undergo regular examinations using hardware diagnostic techniques and take pharmacological medications prescribed by the doctor for life.

Forecast

Hypertrophic cardiomyopathy has different development options. The non-obstructive form is stable, without pronounced symptoms. The consequence of prolonged development of the disease is heart failure. In 5–10% of cases, the thickening of the myocardial wall stops on its own; the same percentage is likely to transform hypertrophic cardiomyopathy into dilated cardiomyopathy (stretching of the heart cavities).

Mortality in the absence of treatment is observed in 3–8% of cases. In half of the patients, sudden death occurs, caused by severe ventricular arrhythmias, complete heart block, or acute myocardial infarction. In 15–25%, coronary atherosclerosis develops. Infectious endocarditis, which affects the mitral and aortic valves, occurs as a complication of the pathology in 9% of patients.

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It is important to know! An effective means for normalization heart function and blood vessel cleansing exists! ...

Despite its prevalence (IVS hypertrophy is observed in more than 70% of people), it is most often asymptomatic and is detected only during very intense physical activity. After all, hypertrophy of the interventricular septum itself is its thickening and the resulting reduction in the useful volume of the chambers of the heart. As the thickness of the cardiac walls of the ventricles increases, the volume of the heart chambers also decreases.

Symptoms and causes of hypertrophic cardiomyopathy

chest pain; shortness of breath with increased physical activity (for example, climbing stairs); dizziness and fainting; increased fatigue; tachyarrhythmia that occurs for short periods of time; heart murmur on auscultation; labored breathing.

The causes of this pathology lie not only in an incorrect lifestyle. Smoking, alcohol abuse, excess weight - all this becomes a factor contributing to the increase in severe symptoms and the manifestation of negative processes in the body with an unpredictable course.

Possible complications of IVS hypertrophy

What complications are possible with the development of cardiopathy of the type under discussion? Everything will depend on the specific case and individual development of the person. After all, many will never know throughout their lives that they have this condition, and some may experience significant physical ailments. We list the most common consequences of thickening of the interventricular septum. So:

1. Heart rhythm disturbances such as tachycardia. Such common types as atrial fibrillation, ventricular fibrillation and ventricular tachycardia are directly associated with IVS hypertrophy. 2. Disorders of blood circulation in the myocardium. Symptoms that occur when the outflow of blood from the heart muscle is disrupted include chest pain, fainting and dizziness. 3. Dilated cardiomyopathy and the associated decrease in cardiac output. The walls of the heart chambers, under conditions of pathologically high load, become thinner over time, which is the cause of the appearance of this condition. 4. Heart failure. The complication is very life-threatening and in many cases ends in death. 5. Sudden cardiac arrest and death.

Of course, the last two conditions are terrifying. But, nevertheless, with a timely visit to the doctor, if any symptom of cardiac dysfunction occurs, a timely visit to the doctor will help you live a long and happy life.

And a little about secrets...

Have you ever suffered from HEART PAIN? Judging by the fact that you are reading this article, victory was not on your side. And of course you are still looking for a good way to get your heart functioning back to normal.

Then read what the experienced cardiologist E.V. Tolbuzina says about this. in his interview about natural ways to treat the heart and cleanse blood vessels.

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Cardiology
Cardiomyopathies
Hypertrophic cardiomyopathy (HCM)

Hypertrophic cardiomyopathy is most often defined as severe hypertrophy of the left ventricular myocardium for no apparent reason. The term “hypertrophic cardiomyopathy” is more precise than “idiopathic hypertrophic subaortic stenosis”, “hypertrophic obstructive cardiomyopathy” and “muscular subaortic stenosis”, since it does not necessarily imply left ventricular outflow tract obstruction, which occurs in only 25% of cases.

Course of the disease

Histologically, hypertrophic cardiomyopathy reveals a disordered arrangement of cardiomyocytes and myocardial fibrosis. Most often, in descending order, the interventricular septum, apex and middle segments of the left ventricle undergo hypertrophy. In a third of cases, only one segment undergoes hypertrophy. The morphological and histological diversity of hypertrophic cardiomyopathy determines its unpredictable course.

The prevalence of hypertrophic cardiomyopathy is 1/500. It is often a familial disease. Hypertrophic cardiomyopathy is probably the most common inherited cardiovascular disease. Hypertrophic cardiomyopathy is detected in 0.5% of patients referred for echocardiography. It is the most common cause of sudden death in athletes under 35 years of age.

Symptoms and complaints

Heart failure

Dyspnea at rest and during exercise, nocturnal attacks of cardiac asthma and fatigue are based on two processes: increased diastolic pressure in the left ventricle due to diastolic dysfunction and dynamic obstruction of the left ventricular outflow tract.

Increased heart rate, decreased preload, shortened diastole, increased left ventricular outflow tract obstruction (eg, with exercise or tachycardia), and decreased left ventricular compliance (eg, with ischemia) aggravate complaints.

In 5-10% of patients with hypertrophic cardiomyopathy, severe systolic dysfunction of the left ventricle develops, dilation and thinning of its walls occurs

Myocardial ischemia

Myocardial ischemia in hypertrophic cardiomyopathy can occur regardless of obstruction of the right ventricular outflow tract.

Myocardial ischemia manifests itself clinically and electrocardiographically in the same way as usual. Its presence is confirmed by myocardial scintigraphy with 201 Tl, positron emission tomography, and increased lactate production in the myocardium with frequent atrial stimulation.

The exact causes of myocardial ischemia are unknown, but it is based on a discrepancy between the need for oxygen and its delivery. The following factors contribute to this.

Damage to small coronary arteries with impairment of their ability to expand.

Increased tension in the myocardial wall, resulting from delayed relaxation in diastole and obstruction of the left ventricular outflow tract.

Decrease in the number of capillaries in relation to the number of cardiomyocytes.

Decreased coronary perfusion pressure.

Fainting and presyncope

Fainting and presyncope occur due to decreased cerebral blood flow as cardiac output falls. They usually occur during exercise or arrhythmias.

Sudden death

The one-year mortality rate for hypertrophic cardiomyopathy is 1-6%. Most patients die suddenly. The risk of sudden death varies from patient to patient. In 22% of patients, sudden death is the first manifestation of the disease. Sudden death most often occurs in older and younger children; It is rare under 10 years of age. Approximately 60% of sudden deaths occur at rest, the rest after heavy physical activity.

Rhythm disturbances and myocardial ischemia can trigger a vicious circle of arterial hypotension, shortened diastolic filling time and increased left ventricular outflow tract obstruction, which ultimately leads to death.

Physical examination

When examining the jugular veins, a pronounced A wave may be clearly visible, indicating hypertrophy and intractability of the right ventricle. A cardiac impulse indicates right ventricular overload and may be noticeable with concomitant pulmonary hypertension.

The apex beat is usually shifted to the left and diffuse. Due to left ventricular hypertrophy, a presystolic apical impulse may appear, corresponding to the IV sound. A triple apical impulse is possible, the third component of which is caused by late systolic bulging of the left ventricle.

The pulse in the carotid arteries is usually bifurcated. A rapid rise in the pulse wave, followed by a second peak, is due to increased contraction of the left ventricle.

Auscultation

The first tone is usually normal, preceded by the IV tone.

The second sound may be normal or paradoxically split due to prolongation of the ejection phase of the left ventricle as a result of obstruction of its outflow tract.

The rough fusiform systolic murmur of hypertrophic cardiomyopathy is best heard along the left sternal border. It is carried out in the area of the lower third of the sternum, but is not carried out on the vessels of the neck and in the axillary region.

An important feature of this noise is the dependence of its volume and duration on pre- and post-load. As venous return increases, the noise shortens and becomes quieter. As the filling of the left ventricle decreases and its contractility increases, the noise becomes rougher and longer lasting.

Tests that influence pre- and afterload make it possible to differentiate hypertrophic cardiomyopathy from other causes of systolic murmur.

Table. The influence of functional and pharmacological tests on the loudness of systolic murmur in hypertrophic cardiomyopathy, aortic stenosis and mitral regurgitation

EDLV - end-diastolic volume of the left ventricle; CO - cardiac output; ↓ - reduction in noise volume; - increase in noise volume.

Mitral regurgitation is common in hypertrophic cardiomyopathy. It is characterized by a pansystolic, blowing noise conducted in the axillary region.

A quiet, diminishing early diastolic murmur of aortic insufficiency is heard in 10% of patients with hypertrophic cardiomyopathy.

Heredity

Familial forms of hypertrophic cardiomyopathy are inherited in an autosomal dominant manner; they are caused by missense mutations, that is, substitutions of single amino acids, in the genes of sarcomeric proteins (see table)

Table. Relative frequency of mutations in familial forms of hypertrophic cardiomyopathy

Familial forms of hypertrophic cardiomyopathy should be distinguished from such phenotypically similar diseases as apical hypertrophic cardiomyopathy and hypertrophic cardiomyopathy of the elderly, as well as from hereditary diseases in which disordered cardiomyocytes and left ventricular systolic dysfunction are not accompanied by hypertrophy.

The least favorable prognosis and the highest risk of sudden death are observed with certain mutations of the myosin heavy beta chain (R719W, R453K, R403Q). With mutations of the troponin T gene, mortality is high even in the absence of hypertrophy. There is not yet enough data to use genetic analysis in practice. The available information relates mainly to familial forms with a poor prognosis and cannot be extended to all patients.

Diagnostics

Although in most cases there are pronounced changes on the ECG (see table), there are no ECG signs pathognomonic for hypertrophic cardiomyopathy.

EchoCG is the best method, it is highly sensitive and completely safe.

The table shows echocardiographic criteria for hypertrophic cardiomyopathy for M-modal and two-dimensional studies.

Sometimes hypertrophic cardiomyopathy is classified depending on the location of the hypertrophy (see table).

Doppler imaging can identify and quantify the effects of anterior systolic motion of the mitral valve.

About a quarter of patients with hypertrophic cardiomyopathy have a pressure gradient in the right ventricular outflow tract at rest; For many, it appears only during provocative tests.

Obstructive hypertrophic cardiomyopathy is defined as an intraventricular pressure gradient of more than 30 mmHg. Art. at rest and more than 50 mm Hg. Art. against the background of provocative tests. The magnitude of the gradient corresponds well to the onset time and duration of contact between the interventricular septum and the mitral valve leaflets; the earlier the contact occurs and the longer it is, the higher the pressure gradient.

If there is no obstruction of the left ventricular outflow tract at rest, it can be provoked by medications (inhalation of amyl nitrite, administration of isoprenaline, dobutamine) or functional tests (Valsalva maneuver, physical activity), which reduce preload or increase contractility of the left ventricle.

The anterior systolic movement of the mitral valve is explained by the suction effect of accelerated blood flow through the narrowed outflow tract of the left ventricle, the so-called Venturi effect. This movement further narrows the outflow tract, increasing its obstruction and increasing the gradient.

Although the clinical significance of left ventricular outflow tract obstruction has been questioned, its surgical or drug removal improves the condition of many patients. Therefore, identifying left ventricular outflow tract obstruction is very important.

Detection of mitral regurgitation and mitral valve changes can significantly influence the tactics of surgical and medical treatment of patients with hypertrophic cardiomyopathy.

Approximately 60% of patients with hypertrophic cardiomyopathy have mitral valve pathology, including excessive leaflet length, calcification of the mitral annulus, and, rarely, abnormal attachment of the papillary muscles to the anterior mitral valve leaflet.

If the structure of the mitral valve is not changed, the severity of mitral regurgitation is directly proportional to the severity of obstruction and the degree of non-closure of the leaflets

The main advantages of MRI for hypertrophic cardiomyopathy are high resolution, absence of radiation and the need for administration of contrast agents, the ability to obtain a three-dimensional image, and assess the structure of the tissue. Disadvantages include high cost, length of examination, and the inability to perform MRI in some patients, such as those with implanted defibrillators or pacemakers.

Cine-MRI allows you to examine the apex of the heart, the right ventricle, and study the functioning of the ventricles.

MRI with myocardial marking is a relatively new method that makes it possible to trace the path of certain points of the myocardium during systole and diastole. This makes it possible to assess the contractility of individual areas of the myocardium and thereby identify areas of predominant damage

Cardiac catheterization and coronary angiography

Cardiac catheterization and coronary angiography are performed to evaluate the coronary artery before myectomy or mitral valve surgery and to determine the cause of myocardial ischemia. Characteristic signs determined during an invasive hemodynamic study are shown in the table and figure.

*Arises as a result of obstruction of the left ventricular outflow tract
**Can occur both due to mitral regurgitation and due to increased pressure in the left atrium.

Even with unchanged coronary arteries, patients may have classic angina. Myocardial ischemia in hypertrophic cardiomyopathy may be based on a sharp increase in myocardial oxygen demand, compression of large coronary arteries by myocardial bridges, as well as systolic compression of the septal branches; in the coronary arteries with hypertrophic cardiomyopathy, reverse systolic blood flow can be determined.

Left ventriculography usually shows a hypertrophied ventricle with a noticeable bulging of the interventricular septum into its cavity, almost complete collapse of the ventricular cavity in systole, anterior systolic movement of the mitral valve and mitral regurgitation. In the apical form of hypertrophic cardiomyopathy, the ventricular cavity takes on the shape of a card peak.

Drawing. Pressure curves in the chambers of the heart in hypertrophic cardiomyopathy
Up. Pressure gradient between the outflow tract (LVOT) and the rest of the left ventricle (LV) Ao - aortic pressure curve
At the bottom. Peak-dome pressure curve in the aorta

Myocardial scintigraphy and positron emission tomography

In hypertrophic cardiomyopathy, myocardial scintigraphy has some features, but in general its importance in diagnosing ischemia is the same as usual. Persistent storage defects indicate scarring after myocardial infarction and are usually accompanied by decreased left ventricular function and poor exercise tolerance. Reversible storage defects indicate ischemia caused by decreased coronary reserve in normal arteries or coronary atherosclerosis. Reversible defects are often silent but appear to increase the risk of sudden death, especially in younger patients with hypertrophic cardiomyopathy.

Isotope ventriculography can reveal delayed filling of the left ventricle and a prolongation of the period of isovolumic relaxation.

Positron emission tomography is a more sensitive method; in addition, it allows one to take into account and eliminate interference associated with signal attenuation.

Fluorodeoxyglucose positron emission tomography confirms the presence of subendocarial ischemia caused by decreased coronary reserve.

Treatment should be aimed at the prevention and treatment of heart failure, which is based on systolic and diastolic dysfunction, arrhythmias and ischemia, as well as the prevention of sudden death.

LVOT - left ventricular outflow tract

Treatment for hypertrophic cardiomyopathy is as varied as its clinical manifestations.

Drug treatment

There is no evidence that beta blockers reduce mortality in hypertrophic cardiomyopathy, however, these drugs are used primarily regardless of the presence of left ventricular outflow tract obstruction.

Beta-blockers eliminate angina, shortness of breath and fainting; According to some reports, their effectiveness reaches 70%. Beta blockers with alpha blocking properties, carvedilol and labetalol, have vasodilatory effects and should probably not be used.

Beta blockers have negative inotropic and chronotropic effects due to suppression of sympathetic stimulation. They reduce myocardial oxygen demand and thereby alleviate or eliminate angina pectoris and improve diastolic filling of the left ventricle, which reduces obstruction of its outflow tract.

Contraindications to beta-blockers are bronchospasm, severe AV block without pacemaker and decompensated left ventricular failure.

Calcium antagonists are second-line drugs. They are quite effective and are used when beta-blockers are contraindicated or when they are ineffective.

Calcium antagonists have a negative inotropic effect and reduce heart rate and blood pressure. In addition, they may improve diastolic function through a rapid filling phase, although they may increase left ventricular end-diastolic pressure.

Apparently, only non-dihydropyrndine calcium antagonists - verapamil and diltiazem - are effective for hypertrophic cardiomyopathy. (see table)

*If well tolerated, doses may be higher.

The effect of calcium antagonists on hemodynamics is unpredictable due to their vasodilatory effect, therefore, in cases of severe obstruction of the left ventricular outflow tract and pulmonary hypertension, they should be prescribed very carefully. Contraindications to calcium antagonists - conduction disorders without pacemaker and systolic
left ventricular dysfunction.

Disopyramide, a Class Ia antiarrhythmic agent, may be prescribed instead of or in addition to a beta blocker or calcium antagonist. Due to its pronounced negative inotropic properties in combination with an antiarrhythmic effect against ventricular and supraventricular arrhythmias, disopyramide is effective for severe obstruction of the left ventricular outflow tract and rhythm disturbances. The disadvantages of disopyramide include its anticholinergic effect, accumulation in the blood during renal or hepatic failure, the ability to improve AV conduction in atrial fibrillation, and the weakening of its antiarrhythmic effect over time. Because of its side effects, disopyramide is usually used only in severe cases, pending more radical treatment: myectomy or destruction of the interventricular septum with ethanol. Disopyramide is not used for long-term treatment of hypertrophic cardiomyopathy.

Non-drug treatment

In very severe patients without left ventricular outflow tract obstruction, the only option is heart transplantation. If there is obstruction and symptoms persist despite drug treatment, dual-chamber pacemaker, myectomy, including mitral valve replacement, and destruction of the interventricular septum with ethanol may be effective.

Dual-chamber pacemaker

The first studies on dual-chamber pacemaker showed that it improves well-being and reduces left ventricular outflow tract obstruction, but these results are now being questioned. The pacemaker may impair ventricular filling and reduce cardiac output. Randomized crossover studies have shown that the improvement is largely due to the placebo effect.

Destruction of the interventricular septum with ethanol

Destruction of the interventricular septum with ethanol is a relatively new method, which is currently being compared with myectomy.

In the cardiac catheterization laboratory, a guidewire is placed in the trunk of the left coronary artery to catheterize the first, second, or both septal branches. A catheter is installed at the mouth of the septal branch, through which an echo contrast agent is injected. This allows you to assess the size and location of a future infarction. A heart attack is induced by injecting 1–4 ml of absolute ethanol into the septal branch.

The akinesia and thinning of the interventricular septum resulting from the procedure reduces or completely eliminates left ventricular outflow tract obstruction. Possible complications include AV block, intimal detachment of the coronary artery, large anterior infarction, and arrhythmias due to post-infarction scar. Long-term results are still unknown.

Surgical treatment of hypertrophic cardiomyopathy

Surgical treatment of hypertrophic cardiomyopathy has been performed for more than 40 years.

In experienced hands, mortality after myectomy (Morrow procedure) does not exceed 1-2%. This operation eliminates the intraventricular gradient at rest in more than 90% of cases; most patients experience long-term improvement. The resulting dilation of the left ventricular outflow tract reduces anterior systolic motion of the mitral valve, mitral regurgitation, left ventricular systolic and diastolic pressure, and intraventricular gradient.

Mitral valve replacement (with low-profile prostheses) eliminates obstruction of the left ventricular outflow tract, but it is indicated mainly for minor septal hypertrophy, after ineffective myectomy, and for structural changes in the mitral valve itself.

Selected issues of diagnosis and treatment

Atrial fibrillation

Atrial fibrillation occurs in approximately 10% of patients with hypertrophic cardiomyopathy and causes severe consequences: shortening of diastole and lack of atrial pumping can lead to hemodynamic disturbances and pulmonary edema. Due to the high risk of thromboembolism, all patients with atrial fibrillation in hypertrophic cardiomyopathy should receive anticoagulants. It is necessary to maintain a low frequency of ventricular contraction; an attempt must be made to restore and maintain sinus rhythm.

For paroxysms of atrial fibrillation, electrical cardioversion is best. To maintain sinus rhythm, disopyramide or sotalol is prescribed; if they are ineffective, amiodarone is used in low doses. With severe obstruction of the left ventricular outflow tract, a combination of a beta-blocker with disopyramide or sotalol is possible.

Persistent atrial fibrillation may be tolerated fairly well if the ventricular rate is kept low with beta blockers or calcium antagonists. If atrial fibrillation is poorly tolerated and sinus rhythm cannot be maintained, destruction of the AV node with implantation of a dual-chamber pacemaker is possible.

Prevention of sudden death

Prophylactic measures such as implantation of a defibrillator or administration of amiodarone (the effect of which has not been proven on long-term prognosis) are possible only after identifying risk factors with sufficiently high sensitivity, specificity and predictive value.

There is no convincing data on the relative importance of risk factors for sudden death. The main risk factors are listed below.

History of circulatory arrest

Sustained ventricular tachycardia

Sudden death of close relatives

Frequent paroxysms of unsustained ventricular tachycardia during Holter ECG monitoring

Recurrent fainting and presyncope (especially during physical exertion)

Decrease in blood pressure during exercise

Massive left ventricular hypertrophy (wall thickness > 30 mm)

Myocardial bridges over the anterior descending artery in children

Left ventricular outflow tract obstruction at rest (pressure gradient > 30 mmHg)

The role of EPI in hypertrophic cardiomyopathy has not been determined. There is no convincing evidence that it allows assessing the risk of sudden death. When performing EPS according to the standard protocol, it is often not possible to induce ventricular arrhythmias in survivors of circulatory arrest. On the other hand, the use of a non-standard protocol can cause ventricular arrhythmias even in patients with a low risk of sudden death.

Clear recommendations for the implantation of defibrillators in hypertrophic cardiomyopathy can only be developed after the completion of appropriate clinical studies. It is currently believed that implantation of a defibrillator is indicated after rhythm disturbances that could result in sudden death, with sustained paroxysms of ventricular tachycardia and with multiple risk factors for sudden death. In the high-risk group, implanted defibrillators are activated in approximately 11% per year among those who have already experienced circulatory arrest, and in 5% per year among those in whom defibrillators were implanted for primary prevention of sudden death.

Sports heart

Differential diagnosis with hypertrophic cardiomyopathy

On the one hand, playing sports with undiagnosed hypertrophic cardiomyopathy increases the risk of sudden death, on the other hand, an erroneous diagnosis of hypertrophic cardiomyopathy in athletes leads to unnecessary treatment, psychological difficulties and unreasonable restrictions on physical activity. Differential diagnosis is most difficult if the thickness of the left ventricular wall in diastole exceeds the upper limit of normal (12 mm), but does not reach the values characteristic of hypertrophic cardiomyopathy (15 mm), and there is no anterior systolic movement of the mitral valve and obstruction of the left ventricular outflow tract .

Hypertrophic cardiomyopathy is supported by asymmetric myocardial hypertrophy, end-diastolic size of the left ventricle less than 45 mm, thickness of the interventricular septum more than 15 mm, enlargement of the left atrium, diastolic dysfunction of the left ventricle, and a family history of hypertrophic cardiomyopathy.

An athletic heart is indicated by a left ventricular end-diastolic dimension of more than 45 mm, an interventricular septal thickness of less than 15 mm, an anteroposterior dimension of the left atrium of less than 4 cm, and a decrease in hypertrophy upon cessation of training.

Exercise for hypertrophic cardiomyopathy

The restrictions remain in place despite medical and surgical treatment.

With hypertrophic cardiomyopathy under the age of 30, regardless of the presence of obstruction of the left ventricular outflow tract, one should not engage in competitive sports that require heavy physical exertion.

After age 30, restrictions may be less stringent because the risk of sudden death likely decreases with age. Sports activities are possible in the absence of the following risk factors: ventricular tachycardia with Holter ECG monitoring, sudden death in close relatives with hypertrophic cardiomyopathy, fainting, intraventricular pressure gradient more than 50 mm Hg. Art., decreased blood pressure during exercise, myocardial ischemia, anteroposterior size of the left atrium more than 5 cm, severe mitral regurgitation and paroxysms of atrial fibrillation.

Infective endocarditis

Infective endocarditis develops in 7-9% of patients with hypertrophic cardiomyopathy. Its mortality rate is 39%.

The risk of bacteremia is high during dental procedures, intestinal and prostate surgery.

Bacteria settle on the endocardium, which is subject to constant damage due to hemodynamic disturbances or structural damage to the mitral valve.

All patients with hypertrophic cardiomyopathy, regardless of the presence of left ventricular outflow tract obstruction, are given antibacterial prophylaxis for infective endocarditis before any interventions accompanied by a high risk of bacteremia.

Apical left ventricular hypertrophy (Yamaguchi disease)

Characterized by chest pain, shortness of breath, and fatigue. Sudden death is rare.

In Japan, apical left ventricular hypertrophy accounts for a quarter of cases of hypertrophic cardiomyopathy. In other countries, isolated apical hypertrophy occurs in only 1-2% of cases.

Diagnostics

The ECG shows signs of left ventricular hypertrophy and giant negative T waves in the precordial leads.

EchoCG reveals the following signs.

Isolated hypertrophy of the sections of the left ventricle located apical to the origin of the chordae tendineae

Myocardial thickness in the apical region is more than 15 mm or the ratio of myocardial thickness in the apical region to the thickness of the posterior wall is more than 1.5

Absence of hypertrophy of other parts of the left ventricle

Absence of left ventricular outflow tract obstruction.

MRI allows you to see limited hypertrophy of the apical myocardium. MRI is used mainly when echocardiography is uninformative.

With left ventriculography, the cavity of the left ventricle in diastole has the shape of a card peak, and in systole its apical part completely collapses.

The prognosis is favorable compared to other forms of hypertrophic cardiomyopathy.

Treatment is aimed only at eliminating diastolic dysfunction. Beta-blockers and calcium antagonists are used (see above).

Hypertensive hypertrophic cardiomyopathy of the elderly

In addition to the symptoms inherent in other forms of hypertrophic cardiomyopathy, arterial hypertension is characteristic.

The exact incidence is unknown, but the disease is more common than one might think.

According to some data, the basis of hypertrophic cardiomyopathy in the elderly is the late expression of the mutant gene for myosin-binding protein C.

Compared to young patients (under 40 years of age), older patients (65 years of age and older) have their own characteristics.

General signs

Intraventricular gradient at rest and during exercise

Asymmetric hypertrophy

Anterior systolic movement of the mitral valve.

Signs common to older people

Less pronounced hypertrophy

Less severe right ventricular hypertrophy

Oval rather than slit-shaped cavity of the left ventricle

Noticeable bulging of the interventricular septum (it takes on an S-shape)

A more acute angle between the aorta and the interventricular septum due to the fact that the aorta unfolds with age

Treatment of hypertrophic cardiomyopathy in the elderly is the same as for its other forms.

The prognosis is relatively favorable compared to hypertrophic cardiomyopathy at a younger age.

Surgical treatment of cardiomyopathies in Belarus - European quality at a reasonable price

Literature
B. Griffin, E. Topol "Cardiology" Moscow, 2008

If asymptomatic, this disease can result in sudden cardiac arrest. It's scary when this happens to seemingly healthy young people who play sports. What happens to the myocardium, why such consequences arise, whether hypertrophy is treated - remains to be figured out.

What is myocardial hypertrophy

chaotic arrangement of muscle fibers;
damage to small coronary vessels;
formation of areas of fibrosis;
obstruction of blood flow - an obstacle to the ejection of blood from the atrium due to displacement of the mitral valve.

increased protein production;
hyperplasia – increase in the number of cells;
increase in myocardial muscle mass;
wall thickening.

Pathological myocardial hypertrophy

growth of capillaries and nerves lags behind;
blood supply is disrupted;
the influence of nervous tissue on metabolic processes changes;
myocardial structures wear out;
the ratio of myocardial sizes changes;
systolic and diastolic dysfunction occurs;
repolarization is disrupted.

Myocardial hypertrophy in athletes

Sports myocardial hypertrophy has 3 types:

eccentric - muscles change proportionally - typical for dynamic activities - swimming, skiing, long-distance running;
concentric hypertrophy - the ventricular cavity remains unchanged, the myocardium increases - observed in gaming and static types;
mixed - inherent in activities with the simultaneous use of immobility and dynamics - rowing, cycling, skating.

Myocardial hypertrophy in a child

heart disease;
myocardial dystrophy;
hypertension;
angina pectoris.

Causes of cardiomyopathy

It is customary to distinguish between primary and secondary causes of hypertrophic development of the myocardium. The first ones are influenced by:

Secondary causes of myocardial hypertrophy are provoked by the following factors:

Left ventricular hypertrophy

the elasticity of the myocardial muscles is lost;
blood circulation slows down;
normal heart function is disrupted;
there is a danger of a sudden load on it.

Right atrial hypertrophy

birth defects;
atrial septal defects, in which blood enters the left and right ventricles simultaneously;
stenosis;
obesity.

Right ventricular hypertrophy is accompanied by symptoms:

hemoptysis;
dizziness;
night cough;
fainting;
chest pain;
shortness of breath without exertion;
bloating;
arrhythmia;
signs of heart failure - swelling of the legs, enlarged liver;
malfunction of internal organs;
cyanosis of the skin;
heaviness in the hypochondrium;
dilation of veins in the abdomen.

Hypertrophy of the interventricular septum

One of the signs of the development of the disease is hypertrophy of the IVS (interventricular septum). The main cause of this disorder is gene mutations. Hypertrophy of the septum provokes:

Dilation of the heart chambers

fast fatiguability;
weakness;
dyspnea;
swelling of the legs and arms;
rhythm disturbances;

Cardiac hypertrophy - symptoms

Forms of cardiomyopathy

basal obstruction – resting state or 30 mm Hg;
latent – calm state, less than 30 mm Hg – it characterizes the non-obstructive form of HCM;
labile obstruction – spontaneous intraventricular gradient fluctuations.

Myocardial hypertrophy - classification

For convenience of work in medicine, it is customary to distinguish between the following types of myocardial hypertrophy:

obstructive – at the top of the septum, over the entire area;
non-obstructive – symptoms are mild, diagnosed by chance;
symmetrical – all walls of the left ventricle are affected;
apical - the heart muscles are enlarged only from above;
asymmetrical - affects only one wall.

Eccentric hypertrophy

interventricular septum;
top;
side wall.

Concentric hypertrophy

Myocardial hypertrophy – degrees

moderate – 11-21;
average – 21-25;
pronounced – over 25.

Diagnosis of hypertrophic cardiomyopathy

At the initial stage, with a slight development of wall hypertrophy, it is very difficult to identify the disease. The diagnostic process begins with interviewing the patient, finding out:

presence of pathologies in relatives;
the death of one of them at a young age;
past diseases;
fact of radiation exposure;
external signs during visual inspection;
blood pressure values;
indicators in blood and urine tests.

A new direction is being used – genetic diagnosis of myocardial hypertrophy. The potential of hardware and radiological methods helps to establish the parameters of HCM:

ECG - determines indirect signs - rhythm disturbances, hypertrophy of sections;
X-ray - shows an increase in the contour;
Ultrasound – assesses myocardial thickness, blood flow disturbances;
echocardiography – records the location of hypertrophy, diastolic dysfunction;
MRI – gives a three-dimensional image of the heart, determines the degree of myocardial thickness;
ventriculography – examines contractile functions.

How to treat cardiomyopathy

Drug treatment of hypertrophic cardiomyopathy includes the use of drugs that:

reduce blood pressure - ACE inhibitors, angiotensin receptor antagonists;
regulate heart rhythm disturbances - antiarrhythmics;
drugs with a negative ionotropic effect relax the heart - beta blockers, calcium antagonists from the verapamil group;
remove fluid - diuretics;
improve muscle strength - ionotropes;
if there is a threat of infective endocarditis, antibiotic prophylaxis.

installation of a defibrillator;
pacemaker implantation;
transaortic septal myectomy;
excision of part of the interventricular septum;
transcatheter septal alcohol ablation.

Cardiomyopathy - treatment with folk remedies

take a spoonful of seeds;
add boiling water - liter;
hold in a water bath for 50 minutes;
filter;
drink per day - dose 100 g.

Oat infusion for regulating the functioning of the heart muscles has good reviews in the treatment of HCM. According to the healers' prescription, it is required:

oats – 50 grams;
water – 2 glasses;
heat to 50 degrees;
add 100 g of kefir;
pour in radish juice - half a glass;
stir, stand for 2 hours, strain;
put 0.5 tbsp. honey;
dosage – 100 g, three times a day before meals;
course – 2 weeks.

Video: hypertrophy of the heart muscle

The information presented in the article is for informational purposes only. The materials in the article do not encourage self-treatment. Only a qualified doctor can make a diagnosis and make recommendations for treatment based on the individual characteristics of a particular patient.

One of the characteristic symptoms of hypertrophic cardiomyopathy is hypertrophy of the IVS (interventricular septum). When this pathology occurs, thickening of the walls of the right or left ventricle of the heart and the interventricular septum occurs. This condition itself is a derivative of other diseases and is characterized by an increase in the thickness of the walls of the ventricles.

In practice, this all leads to a reduction in the volume of blood that is released by the heart into the vascular bed of the body. To provide the organs with a normal amount of blood under such conditions, the heart must contract stronger and more often. And this, in turn, leads to its early wear and tear and the occurrence of diseases of the cardiovascular system.

A large number of people around the world live with undiagnosed IVS hypertrophy, and only with increased physical activity does their existence become known. As long as the heart can ensure normal blood flow to the organs and systems, everything proceeds hidden and the person will not experience any painful symptoms or other discomfort. But you should still pay attention to some symptoms and contact a cardiologist if they occur. These symptoms include:

chest pain;
shortness of breath with increased physical activity (for example, climbing stairs);
dizziness and fainting;
increased fatigue;
tachyarrhythmia that occurs for short periods of time;
heart murmur on auscultation;
labored breathing.

It is important to remember that undiagnosed IVS hypertrophy can cause sudden death even in young and physically strong people. Therefore, a medical examination by a therapist and/or cardiologist should not be neglected.

And doctors call gene mutations the reason for the development of IVS thickening. As a result of these changes at the level of the human genome, the heart muscle becomes abnormally thick in some areas.

The consequences of the development of such a deviation become dangerous.

After all, additional problems in such cases will be disturbances in the conduction system of the heart, as well as weakening of the myocardium and the associated decrease in the volume of blood ejected during cardiac contractions.