Moderate myocardial hypertrophy MZP. Obstructive cardiomyopathy

Hypertrophy of the left or right hearts of the heart occurs due to the damage of the muscle, the valves of the organ, due to the blood circulation violation. Often this happens with congenital anomalies of development, due to the increase in blood pressure, diseases of the lungs, significant physical exertion. Most often stated hypertrophy of the left ventricle heart. This is due to a greater functional load in this area.

Causes of appearance

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Causes of appearance

The disease occurs as a result of various violations that impede the normal operation of the organ. Myocardia begins to shrink with an increased load, it increases metabolism, the volume of tissue and the mass of cells increases.

At the initial stage of the disease, the heart maintains normal bloodstream due to the increase in its mass. But in the future myocardium is depleted, and hypertrophy is replaced by atrophy - cells are significantly reduced in size.

There are two types of pathology: concentric - the heart increases, its walls are thicken, the atrium / ventricles are reduced, and the eccentric (the organ is increased, but the cavities are expanded).

Heart hypertrophy may affect healthy people engaged in physical labor and athletes. Against the background of such changes may occur with acute heart failure. By studying bodybuilding, hockey, severe physical labor, you need to follow the state of myocardium.

Due to the occurrence of ventricular hypertrophy, divided into 2 types:

• working - due to increased load on a healthy body;
• the replacement is the result of the device to work with a different disease.

Causes of lesions of the left ventricle

Most often, changes are subject to the muscle of the left ventricle. If its thickness is greater than 1.2 cm, this violation takes place. At the same time, hypertrophy of the MZHP (interventricular septum) of the heart is observed. In severe cases, the thickness can reach 3 cm, and the weight is 1 kg.

Blood blood pumping in the aorta is provoked, so blood supply to the whole body is disturbed. An increase in mass leads to a lack of oxygen and nutrients. As a result, hypoxia and sclerosis occurs.

Causes of left ventricular changes: arterial hypertension; cardiomyopathy; narrowing (stenosis) of the aortic valve; increased physical exertion; hormonal disorders; obesity; Kidney disease with secondary hypertension.

Causes of leaving the left atrium:

• Arterial hypertension;
• Hypertrophic cardiomyopathy;
• Congenital heart / aortic pathology;
• Common obesity, especially in children and adolescents;
• Stenosis / lack of aortic or mitral valve.

Causes of the defeat of the right ventricle

Changes in the right of atrium are usually associated with pulmonary pathologies and a violation in a small circle of blood flow. In the right atrium, blood on the hollow veins comes from tissues and organs. From there she gets into the ventricle through a three-rolled valve and on, into the pulmonary artery and lungs.

In the latter there is gas exchange. It is for this reason that the normal structure of the right departments is disturbed due to the various diseases of the respiratory organs.

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

• Congenital development pathology (Tetradafallo Ave, MZP Defect);
• Chronic obstructive lung diseases, for example, emphysema, pneumosclerosis, bronchial asthma, bronchitis;
• Stenosis / deficiency of a three-range valve, changes in the pulmonary artery valve, an increase in the right ventricle.

Chronic pathology of the lungs provokes the damage to the vessels of a small circle, the growing of the connecting tissues, gas exchange and microcircular channel decreases. As a result, blood pressure increases in the lung vessels, so myocardium begins to decline with greater force, which leads to hypertrophing.

The narrowing or incomplete closure of the tricuspid valve leads to the same blood flow impaired, as in a similar case in the pathology of mitral.

Causes of changes in the right ventricle: Congenital malformations, chronic lung hypertension, narrowing of the pulmonary artery valve, increase the venous pressure during stagnation of failure.

Hypertrophy of the right ventricle of the heart takes place if the thickness of its wall is greater than 3 mm. It leads to expansion of departments and bad blood circulation. As a result, the venous return on the hollow veins is violated, stagnation appears. Patients appear swelling, shortness of breath, skin sinusiness, and then complaints about the work of the internal organs.

It should be noted that with the lesion of the left ventricle, the left atrium will suffer. Then the changes are subject to changes.

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

With the lesion of myocardial, the left half arise: fainting, dizziness, shortness of breath, arrhythmias, pain in this area, weakness, fast fatigue.

With the defeat of the right-handed, the following symptoms arise: cough, shortness of breath, difficulty breathing; swelling; cyanosis, pale leather; Violation of rhythm.

How to diagnose hypertrophy of both ventricles of the heart

The simplest and at the same time effective methods is an ultrasound study (ultrasound) and echocardiography (ECG). In the process, the wall thickness and body size is determined.

Indirect symptoms of changes detected by ECG:

• When changing the right departments, electrical conductivity changes, the rhythm is disturbed, the deviation of the electric axis is observed;
• Developments in the left sections indicate the deviation of the axis to the left, respectively, lintels are recorded.

Confirm or refute the diagnosis is also possible according to the results of X-ray of the organs of the chest.

Treatment of different forms of heart hypertrophy

All efforts to eliminate the disease are guided primarily for the cause, which caused it.

For example, in the event of a violation due to the disease of the respiratory organs, the course of treatment is sent to compensate for the lung function. Anti-inflammatory therapy is prescribed. Apply bronighting drugs and a number of others depending on the root causes.

Under the defeat of the left departments caused by arterial hypertension, treatment assumes only the reception of hypotensive medicines of different groups, as well as diuretics.

If pronounced valve defects are found, they can resort to surgical interference and even prosthetics.

Treatment of the left and right ventricular heart hypertrophy in all cases of the disease involves the elimination of symptoms of myocardial lesions. It uses antiarrhythmic therapy, as well as cardiac glycosides.

Perhaps prescribe drugs that improve the process of metabolism in the heart muscle (Riboxin, ATP, etc.). Patients are advised to adhere to a special diet, limit the consumption of fluid and salt. In obesity, efforts are directed to normalize body weight.

With congenital heart defect, pathology eliminate surgically, if possible. In very difficult cases, when the structure is very violated and hypertrophic cardiomyopathy develops, the only exit is the organ transplant.

As one can judge from the above, the approach to the patient is purely individually. Doctors take into account all the available manifestations of organ impairments, the overall condition of the patient, the presence of concomitant diseases.

It should be noted that on time the detected myocardial pathology in the overwhelming majority of cases can be corrected. Feeling the first alarming symptoms need to immediately apply for a consultation to a specialist - cardiologist. After the examination, it will reveal the cause of the disease and prescribe adequate treatment.

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The reasons

The causes of hypertrophic cardiomyopathy were established after the ultrasound of the relatives of patients. It turned out that similar changes in the heart muscle are available in 65% of the members of one family.

There are 2 forms of the disease according to etiological sign.

Primary or idiopathic

Primary called the hereditary form of cardiomyopathy. The development of genetics made it possible to establish an accurate gene responsible for the development of the disease in half cases. In 50% of families of accurate instructions on modified genes are not established.

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

Scientists believe that the mutation of genes can occur under the influence of such a non-malfunction in the external environment (smoking, suffering infections, radiation), acting on the future mother during pregnancy.

Secondary

Secondary changes are formed after 60 years in patients with hypertension, which had changes in the structure of muscle tissue in the intrauterine period.

It has been established that 1/5 patients who lived to elderly can develop the weakness of systole and the expansion of the left ventricular cavity. In such cases, hypertrophic cardiomyopathy is not different from the dilatation form.

The mechanism of development of pathology

As a result of genetic mutations in muscle tissue, "incorrect" main protein molecules appear, providing the reduction process, actin and myosin. They do not produce due calories due to a sharp decline in the content of the necessary enzymes. In 90% of patients, muscle cells lose their focus. In myocardial tissue, areas are formed not capable of reduced.

In response, other fibers take on operating functions. Their muscle mass increases (hypertrophy), because it has to be reduced with an increased load. The thickness of the left ventricle increases, although no data on congenital and acquired vices and hypertension is available. At the same time there is a thickening of the interventricular partition. This leads to narrowing blood ejection paths in the aorta.

Plots of hypertrophy can be located foci (more often at the outlet of the aorta) or to strive most of the left ventricle. Less often apply to the right departments of the heart muscle. There is damage to the valve valves (mitral and aortic), vessels that feed myocardium.

During the diastole, atrial studies have to work with a reinforced load to fill the ventricles, because the tissues become dense, solid, lose elasticity. The pressure in the pulmonary circulation of blood circulation is growing.

Increased muscular weight requires more oxygen. The inconsistency of the growth of myocardial requests with opportunities leads to the development of ischemia. This also contributes to the mechanical compression of the mouth of the left corneous artery.

Types of heart lesions

In connection with the uniformity and symmetricality of the development of areas of myocardial hypertrophy, such forms are distinguished:

• symmetric (concentric) - the thickness of the left ventricle walls on the front, rear surfaces and in the partition area, the hypertrophy of the right ventricle is added less increasing; hypertrophy of the right ventricle is added less often;
• asymmetric - plots of thickening are formed in the upper or lower part of the interventricular septum, it becomes one and a half or three times the thicker of the back wall of the left ventricle (in the normal heart they are equal), in 2/3 of patients these changes are combined with hypertrophy of the front, side walls of the left ventricle or tops, without changes in the back wall.

According to the strength of the thread of blood from the left ventricle in the aorta, it is customary to distinguish:

• obstructive hypertrophic cardiomyopathy (subaportal or sublined) - a change in the anatomical relationships creates a barrier for blood output;
• unstructive - there is no barrier.

Clinical picture

Symptoms of hypertrophic cardiomyopathy appear for the first time in 20 - 25 years. The most characteristic are as follows:

• Pains for chest godic character, very similar to the attacks of angina, have similar irradiation in the left shoulder, neck, shovel. Unlike angina, they are not removed with drugs containing nitroglycerin. There are atypical pain or stitching type.


• An important feature is an increase in shortness of breath when changing the horizontal position of the body to vertical. Over time, the increase in shortness of breath leads to cardiac asthma and lung edema.
• Arrhythmias, acceleration of heartbeat.
• Dizziness, reaching a fainting state, is associated with a violation of the brain power. Intensifying with exercise, outraging, after abundant food, with a rapid upside down.

For hypertrophic cardiomyopathy, a characteristic manifestation is a sudden death death (in the classification it is clarified that since the loss of consciousness should not pass more than 1 hour, the case cannot have any signs of violence).

How to reveal the disease

Diagnosis of the disease is very complex. The doctor needs to know the family history (cases of confirmed disease in relatives or sudden death in young age), the course of pregnancy at the mother, connection with industrial poisonous substances transferred infectious diseasesStaying in areas with high radiation.

When examining, the doctor draws attention to the pallor of the skin, lip sinusiness, fingers. Increased or normal blood pressure is recorded.

Auscultivically listens to the characteristic noise over the projection of the aorta.

In order to eliminate the possible pathology of the heart and blood vessels, a general blood test is checked, urine, biochemical tests for the metabolic products, glucose, blood coagulating system.

Additional examination methods

Hardware diagnostics allows you to accurately identify the problems of the disease.

• The ECG study records information on the disturbed rhythm, hypertrophy of the department of the heart, the development of the blockade.
• On the phonogram, noise is recorded from certain points, which allows you to establish the connection of the listened noise with the aorta.
• The X-ray shown an increase in the circuits of the heart shadow, but the dimensions can be normal if hypertrophy develops inside the cavity.
• Ultrasound is the main method in diagnosis. The size of the heart chambers, the thickness of the walls, the state of the valve apparatus, the interventricular partition is estimated, the blood flow is observed.
• Magnetic resonance tomography allows you to get a three-dimensional image of the heart, reveal the obstruction, the degree of wall thickness.
• Genetic studies - the method of the future, as long as they are not developed.
• Using the introduction of catheters in the cavity of the heart is studied and the pressure of the atrial and ventricles is measured, the rate of blood flow. The technique allows you to take the material on the biopsy.
• The coronary of the heart vessels is carried out by patients after 40 years for differential diagnosis with ischemic lesions of the heart vessels.

Biopsy is allowed only with the exclusion of all other diseases and the absence of help from other diagnostic methods. Under the microscope, modified muscle fibers become visible.

Treatment

Specific elimination of gene mutations can not be achieved. Treatment of hypertrophic cardiomyopathy is carried out by medicines affecting all sides of the pathogenesis of the disease.

When identifying signs of the disease, it is necessary to limit physical exertion, stop sports.

If the patient has any chronic infectious diseases, is carried out preventive purpose Antibiotics.

Groups of drugs that block adrenoreceptors, calcium antagonists are used, funds that reduce the thrombosis in the cavities of the heart are added.

Surgical methods

The method of choice on the open heart is Motomy - removal of part of the interventricular partition from the inside or through the aorta. Mortality in these operations reaches 5%, which is comparable with overall death.

A more gentle technique is carried out - concentrated alcohol is introduced through the puncture of the chest and heart under the control of the ultrasound in the partition area. Artificially caused the death of cells, the thinning of the partition. The obstacle for the passage of blood decreases.

For the treatment of impaired rhythm, an electrostimulator or defibrillator is implanted (depending on the type of violations).

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

When obstruction, an operation to replace the mitral valve to artificially applies, this eliminates its contact with the partition and "clears" the passage for blood flow.

The course of the disease

Hypertrophy is possible from the moment of birth. But most patients, she begins to manifest in adolescence. For three years, the thickness of the wall of myocardium increases by 2 times. At the same time, symptoms of the disease do not detect in 70% of patients. By 18 years (less often to 40), the progression of the thickening of the wall of the heart stops.

In the future, clinical manifestations are formed during the obstructive version of pathology. In cases of unstructive forms, a favorable and is revealed by chance with an ECG examination.

The frequency of annual sudden death from hypertrophic cardiomyopathy and its complications among the adult population - up to 3%, among children - from 4 to 6%. The main reason is the fibrillation of ventricles.

What complications are possible

Hypertrophic cardiomyopathy does not proceed isolated, the disease affects all sides of the heart activity, causes serious complications.

• Arrhythmias and disturbed conductivity are observed almost every patient. Depending on gravity, they can go first in the threat of life to the patient. They are the immediate cause of stopping the heart or fibrillation.
• The attachment of infection of mitral and aortic valves leads to the development of endocarditis, followed by deficiency of the valve apparatus.
• The tomb of the thrombus and the enclosure of the embol in the brain vessels (up to 40% of cases), in the internal organs, in the artery of the limbs occurs during cercerative arrhythmia, paroxysmal form.
• The development of chronic heart failure is possible with a long-term course of the disease, when part of the muscle fibers of myocardium is replaced by a scar cloth.

Forecast

Treatment can lead to temporary stabilization of hypertrophy. The average life expectancy does not depend directly on the form of the disease. The most favorable is the forecast with a long asymptomatic flow, as well as with the top localization and the absence of cases of sudden death among relatives.

The main feature that aggresses the prognosis among patients from 15 to 50 years old, fainting is considered to identify on ECG ischemia, ventricular tachycardia. The appearance of patients with shortness of breath and painful pain sharply increases the risk of sudden death.

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

Difficulties in the etiological factors of the disease make practically impossible any prevention. With this pathology, the main attention should be paid to identifying, starting with adolescence, conducting symptomatic therapy.

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What is myocardial hypertrophy

This is an autosomal dominant disease, hersic signs of gene mutation is striking the heart. It is characterized by an increase in the thickness of the stomach walls. Hypertrophic cardiomyopathy (GKMP) has a classification code on the ICD 10 No. 142. The disease is more likely to be asymmetric, the left ventricle of the heart is more susceptible. At the same time takes place:

• chaotic arrangement of muscle fibers;
• damage to small coronary vessels;
• education of fibrosis sites;
• obstruction of blood flow - an obstacle to the release of blood from the atrium due to the displacement of the mitral valve.

With high loads on myocardium caused by diseases, sports, or harmful habits, the body's protective reaction begins. It is necessary to cope with the overwhelmed volumes of work without increasing the load per unit of mass. Compensation begins to occur:

• strengthening protein generation;
• hyperplasia - the increase in the number of cells;
• increase muscular mass myocardium;
• thickening the wall.

Pathological hypertrophy of myocardium

With long-term work of myocardium under loads that are constantly improved, the pathological form of GKMP occurs. The hypertrophied heart is forced to adapt to new conditions. Myocardial thickening occurs with a rapid pace. With this position:

• lagging behind the growth of capillaries and nerves;
• blood supply is broken;
• the effect of nervous tissue on metabolic processes is changing;
• the structures of myocardium are wearing;
• the ratio of myocardial size changes;
• systolic, diastolic dysfunction arises;
• repolarization is disturbed.

Myocardial hypertrophy in athletes

The abnormal development of myocardium is inconspicuous - hypertrophy - at the athletes. With high physical stress, the heart pumps large volumes of blood, and the muscles adapting to such conditions increases in size. Hypertrophy becomes dangerous, provokes stroke, heart attack, sudden heart stop, in the absence of complaints and symptoms. You can not sharply throw training so that there are no complications.

Sports hypertrophy of myocardium has 3 types:

• eccentric - muscles are changed proportionally - characteristic of dynamic training - swimming, skis, running to large distances;
• concentric hypertrophy - remains without change the cavity of the ventricles, myocardium increases - is noted during gaming and static species;
• mixed - inherent classes with simultaneous use of immobility and speakers - rowing, bike, skates.

Myocardial hypertrophy in a child

The appearance of myocardial pathologies from the moment of birth is not excluded. Diagnosis at such age is difficult. It is often observed hypertrophic changes in myocardium in the adolescence, when cardiomyocythic cells are actively growing. The thickening of the front and rear walls occurs up to 18 years, then stops. The hypertrophy of the ventricles in the child is not considered a separate disease - this is the manifestation of numerous ailments. Children with GKMP often have:

• heart disease;
• myocardial dystrophy;
• hypertensive disease;
• angina.

Causes of cardiomyopathy

It is customary to share the primary and secondary causes of the hypertrophic development of myocardium. The first is influenced by:

• viral infections;
• heredity;
• stress;
• alcohol consumption;
• physical overload;
• excess weight;
• toxic poisoning;
• changes in the body during pregnancy;
• drug use;
• disadvantage in the organism of trace elements;
• autoimmune pathology;
• malnutrition;
• smoking.

Secondary causes of myocardial hypertrophy provoke such factors:

Hypertrophy of the left ventricle heart

More than hypertrophy are subject to walls in the left ventricle. One of the reasons for the GLF is a high pressure that makes myocardium work in an accelerated rhythm. Due to the arising overloads, the left ventricular wall and the MZhP increase in size. With such a situation:

• the elasticity of myocardial muscles is lost;
• blood circulation slows down;
• violates normal heart work;
• there is a danger of a sharp load on it.

Cardiomyopathy left ventricle increases the need of heart in oxygen, nutrients. It is possible to notice the changes in the GLF with a tool examination. A small emission syndrome appears - dizziness, fainting. Among the signs accompanying hypertrophy:

• angina;
• pressure drops;
• heartache;
• arrhythmia;
• weakness;
• high blood pressure;
• bad state of health;
• shortness of breath at rest;
• headache;
• fatigue;

Hypertrophy of the right atrium

An increase in the wall of the right ventricle is not a disease, but pathology that appears when overloading in this department. It occurs due to the receipt of a large number of venous blood from large vessels. The cause of hypertrophy may be:

• congenital vices;
• defects of the interproveserving partition, in which blood gets at the same time in the left and right ventricle;
• stenosis;
• obesity.

Hypertrophy of the right ventricle is accompanied by symptoms:

• hemochlorin;
• dizziness;
• night cough;
• fainting;
• chest pain;
• shortness of load;
• bloating;
• arrhythmia;
• signs of heart failure - foot swelling, liver increase;
• malfunctions of the internal organs;
• skin sinusiness;
• heaviness in the hypochondrium;
• expansion of veins on the stomach.

Hypertrophy of the interventricular partition

One of the signs of the development of the disease - hypertrophy of the MZP (interventricular partition). The main reason for this violation is the mutation of genes. Hyperence hypertrophy provokes:

• ventricular fibrillation;
• flickering arrhythmia;
• problems with the mitral valve;
• ventricular tachycardia;
• violation of blood outflow;
• heart failure;
• stop the heart.

Dilation of heart chambers

Hypertrophy of the interventricular partition can provoke an increase in the internal volume of cardiac cameras. Such an extension is called the dilatation of myocardium. In this position, the heart cannot perform the function of the pump, the symptoms of arrhythmia, heart failure arise:

• fast fatiguability;
• weakness;
• dyspnea;
• swelling feet and hands;
• rhythm disorders;

Heart hypertrophy - symptoms

Danger of myocardial disease in asymptomatic flowing for a long time. It is often diagnosed by chance with medical examinations. In the development of the disease, signs of myocardial hypertrophy may be observed:

• pain in the chest;
• heart rate violation;
• shortness of breath at rest;
• fainting;
• fatigue;
• labored breathing;
• weakness;
• dizziness;
• drowsiness;
• edema.

Cardiomyopathy forms

It should be noted that the disease characterize three forms of hypertrophy, taking into account the systolic pressure gradient. All together corresponds to the obstructive form of GKMP. Allocated:

• basal obstruction - a state of rest or 30 mm mercury pillar;
• latent - a calm condition, less than 30 mm mercury pillars - the unstructive form of GKMP is characterized;
• labyl obstruction - spontaneous intraventricular oscillations of the gradient.

Myocardial Hypertrophy - Classification

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

• obstructive - at the top of the partition, throughout the area;
• unstructive - symptoms are weakly pronounced, diagnosed by randomly;
• symmetric - all the walls of the left ventricle are affected;
• upper - the muscles of the heart are increased only on top;
• asymmetric - only one wall affects.

Eccentric hypertrophy

In this form, the GLF occurs the expansion of the ventricle cavity and at the same time uniform, proportional sealing of myocardial muscles caused by the growth of cardiomyocytes. With a general increase in the mass of the heart, the relative wall thickness remains unchanged. Eccentric myocardial hypertrophy may affect:

• interventricular partition;
• top;
• side wall.

Concentric hypertrophy

For the concentric type of disease, the preservation of the inner cavity is characterized by increasing the mass of the heart due to a uniform increase in the wall thickness. There is another name of this phenomenon - symmetric myocardial hypertrophy. There is a disease as a result of hyperplasia by organlel of myocardiocytes, provoked by high blood pressure. Such development of events is characteristic of arterial hypertension.

Myocardial hypertrophy - degree

To properly estimate the patient's condition for the disease, a special classification was introduced, taking into account myocardial thickening. By how increased the size of the walls during the reduction of the heart, 3 degrees are distinguished in cardiology. Depending on the thickness of 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 minor development of hypertrophy of the wall, identify the disease very difficult. The process of diagnosis begins with a patient's survey, clarification:

• the presence of pathologies in relatives;
• the death of some of them in young age;
• transferred diseases;
• the fact of radiation exposure;
• external signs with visual inspection;
• arterial pressure values;
• indicators in blood tests, urine.

Finding an application A new direction is the genetic diagnosis of myocardial hypertrophy. Helps to establish the parameters of the GKMP potential of hardware and radiological methods:

• ECG - determines indirect signs - rhythm disorders, hypertrophy of departments;
• x-ray - shows an increase in the contour;
• Ultrasound - assesses the thickness of myocardium, violation of blood flow;
• echocardiography - fixes the place of hypertrophy, violation of diastolic dysfunction;
• MRI - gives a three-dimensional image of the heart, sets the degree of myocardine thickness;
• ventriaculture - Explore contractual functions.

How to treat cardiomyopathy

The main purpose of treatment is the return of myocardium to optimal sizes. Events aimed at this are carried out in the complex. Hypertrophy can be cured when an early diagnosis is made. An important part in the myocardium recovery system plays a lifestyle that implies:

• compliance with diet;
• refusal of alcohol;
• cessation of smoking;
• weight loss;
• exclusion of drugs;
• restriction of salt consumption.

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

• reduce pressure - aPF inhibitors, angiotensin receptor antagonists;
• regulate heart rate disorders - antiarrhythmics;
• relaxed heart preparations with negative ionotropic effect - beta blockers, calcium antagonists from verapamil group;
• remove liquid - diuretics;
• improve muscle strength - ionotrops;
• in the threat of infectious endocarditis - antibioticoprophylaxis.

The effective method of treatment, changing the progress of excitation and reduction of ventricles - two-chamber electrocardialism with a shortened atrioventricular delay. More complex cases - pronounced asymmetric hypertrophy of MZP, latent obstruction, lack of effect from the drug - require surgeons to regress. Save life to the patient help:

• installation of the defibrillator;
• cardiac implantation;
• cryportal septal mestectomy;
• excision of part of the interventricular partition;
• transcatheter septal alcohol ablation.

Cardiomyopathy - treatment of folk remedies

On the recommendation of the attending cardiologist, you can supplement the main course of plant reception. The national treatment of left ventricular hypertrophy involves the use of viburnum berries without heat treatment at 100 g per day. It is useful to use flax seeds positively acting on the cells of the heart. Recommend:

• take a spoonful of seeds;
• add boiling water - liter;
• hold 50 minutes on a water bath;
• filter
• to drink in the day - the dose of 100 g.

Good reviews have in the treatment of GKMP oat infusion to regulate the muscles of the heart. By recipe healers are required:

• oats - 50 grams;
• water - 2 glasses;
• warm up to 50 degrees;
• add 100 g kefir;
• pour radish juice - half a cup;
• stir, withstand 2 hours, strain;
• put 0.5 tbsp. honey;
• dosage - 100 g, three days a day before meals;
• course - 2 weeks.

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Definition. R "ROURGSђС,С,СђРС "С" Rysџ myocardium left ventricle (GML) - excess of the mass of the left ventricle relative due to the thickening (growth) of myocardium (heart muscle).

Methods for diagnosing GML. Currently, 3 instrumental techniques are used to diagnose GMLS:

— Standard ECG. When verifying the GML, the usual ECG as a whole differs in low sensitivity - no more than 30%. In other words, of the total number of patients who objectively have GMLs, ECG allows you to diagnose it only in a third. However, the more pronounced hypertrophy, the higher the probability to recognize it through the usual ECG. Heavy hypertrophy practically has ECG markers. Thus, if the GMLG is correctly diagnosed correctly, then it most likely indicates its serious degree. Unfortunately, in our medicine, ordinary ECG gives too much importance in the diagnosis of GML. Often, using low-speed ECG criteria GMLs, doctors affirmatively talking about the phenomenon of hypertrophy where it really is not. Do not expect from the standard ECG more than it can actually show.

— Ultrasound of the heart. It is the "gold standard" in the diagnosis of GML, because it allows real-time to visualize the walls of the heart, and perform the necessary calculations. To estimate myocardial hypertrophy, it is customary to calculate relative values \u200b\u200breflecting the mass of myocardium. However, for simplicity, it is permissible to know the magnitude of only two parameters: the thickness of the front (interventricular septum) and the rear wall of the left ventricle, which allows diagnosing hypertrophy and its degree.

— Magnetic resonance Tomography (MRI). Expensive method of layered scan "Zone of interest". To assess the GML used only if for some reason it is not at the ultrasound of the heart: for example, a patient with obesity and emphysema light heart will be closed on all sides pulmonary clothThat will make it an ultrasound visualization of the impossible (extremely rare, but this is found).

To the myocardial hypertrophy of the left ventricle, the thickness of the MZHP and ZSUL (the clinical value of the CDR with hypertrophy will be said РѕРёР¶Рμ). If the normal value is exceeded, even one of the two parameters presented, it is legally talking about "hypertrophy".

Causes and pathogenesis GML. Clinical conditionsthat can lead to GML (in order to reduce the frequency of occurrence):

1. Diseases leading to an increase in post-load on the heart:

— Arterial hypertension (hypertension, secondary hypertension)

— Pulk heart (congenital or acquired) - aortic stenosis.

By postgroup, they understand the combination of the physico-anatomical parameters of the cardiovascular organism, creating an obstacle to the passage of blood by arteries. Postgroup is determined mainly by a tone peripheral arteries. A certain basic value of the tone of the arteries is the norm and one of the bonded manifestations of homeostasis supporting the level of blood pressure, according to the current needs of the body. Excessive strengthening of the tone of the arteries will mark the increase in post-loading, which is clinically manifested by an increase in blood pressure. So, in the peripheral arterial spa, the load on the left ventricle increases: it needs to be reduced harder to "push the blood through the narrowed artery. This is one of the main stars of pathogenesis in the formation of a "hypertonic" heart.

The second common cause leading to an increase in post-loading on the left ventricle, which means that the obstacle is arterial blood flow - aortic stenosis. In the aortal stenosis, aortic valve is affected: it is wrinkled, calcined, deformed. As a result, the aortal hole becomes so small that the left ventricle must be reduced much stronger to ensure the passage of adequate blood volume through a critically narrow place. Currently, the main cause of the aortic stenosis is a sedenive (senile) damage to the valve in people of old age.

Microscopic changes in myocardial hypertrophy are in the thickening of cardiac fibers, in some sprouting of the connective tissue. At first, it is compensatory, however, with a long existing increased postload (for example, with many years of untreated hypertension), hypertrophied fibers are subjected to dystrophic changes, the architectonics of myocardial sycytosis is disturbed, sclerotic processes in myocardium are predominant. As a result, hypertrophy from the compensation phenomenon turns into a cardiac insufficiency manifestation mechanism - the heart muscle cannot work with a voltage without consequences.

2. Congenital reason GML: hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy is a genetically determined disease, which is characterized by the appearance of unmotivated GML. The manifestation of hypertrophy occurs after birth: as a rule, in children's or adolescence, less often in adults, but in any case no later than 35-40 years. Thus, with hypertrophic cardiomyopathy, the GML arises against the background of complete well-being. This disease is not very rare: according to statistics they suffer from 1 person out of 500. In its clinical practice, I meet 2-3 patients with hypertrophic cardiomyopathy every year.

In contrast to the hypertensive heart in hypertrophic cardiomyopathy, the GML can be very pronounced (severe) and often asymmetric (about it. Read more Rosroor¶rμ). Only with hypertrophic cardiomyopathy the thickness of the wall of the left ventricle reaches sometimes "proceedable" values \u200b\u200bof 2.5-3 cm and more. Microscopically, the architectonics of heart fibers is roughly broken.

3. GML, as a manifestation of system pathological processes.

— Obesity. Excessive body weight is not exceptionally a cosmetic problem. This deep pathophysiological process affecting all organs and systems in which biochemical processes are changing, psychodynamics of thinking, human self, etc. In obesity, the fatty tissue in excess is postponed not only under the skin, but also in almost all organs. The heart is forced to ensure blood "body with all its excessive mass." Such an increased load cannot but affect the heart functionality - it is definitely increasing: the heart is reduced more often and stronger. Thus, when obesity, the GML can develop in the absence of a persistent arterial hypertension.

In obesity, myocardia is thickened not only due to the growth of cardiac fibers and connective tissue, but also due to the deposition of excess fat.

— Amyloidosis (primary or secondary) - pathology, in which a special amyloid protein is postponed in the internal organs, leading to the development of diffuse sclerosis and deficiency of organs. With all the possibility of developing the GML due to amyloidosis, it rarely goes to the fore in the clinic of the disease: other organs (for example, kidneys) are more affected, which will determine the specific picture of the disease.

4. Regarding the natural causes of GML.

— Elderly age. Senile age It is characterized by slow but steadily progressive degradation (dystrophy) of all organs and systems. Decreases the proportion of water and the parenchymal components in the organs; On the contrary, sclerotic processes are enhanced. The old man's heart is no exception: muscle fibers are thinned, breaking down, at the same time, the connecting tissue is strongly developing, due to which the GMLG is preferably and arises in old age. What is important to know, senile GML, in the absence of other reasons, never reaches significant values. It does not exceed the degree of "insignificant" and more often is only an age phenomenon, without having a special clinical value.

— Heart athlete. We are talking about persons who have been engaged in professional sports for a long time. Gymnges in such subjects can be called in a pure form of compensatory (working), as well as the accompanying hypertrophy of skeletal muscles. After the end of the sports career, the GMLG is subjected to full or partial regression.

The following diseases lead to concentric GMLs:

S-hypertrophy does not have a special clinical value, which is more often a marker of the "age" heart. Occasionally, this type of hypertrophy occurs in middle-aged people.

The clinical value of the GML. Diseases leading to the development of GMLs can for a long time (years, decades) to proceed asymptomatic or to have non-specific manifestations: for example, headache with arterial hypertension. The earliest symptom of the GML (who, by the way, may appear years after the existence of hypertrophy) is dyspnea With a familiar exercise: walking, climbing the stairs. Srunning mechanism: РёР ° СѓС,РѕР »РёР ‡ РμСѓРєР ° Сџ СѓРμСђРґРμС ‡ РѕР ° Сџ РѕРμРґРѕСѓС,Р ° С,РѕС З РѕРѕСѓС Сњ. It is known that the blood blood flow occurs during the diastole (relaxation): the blood in the concentration gradient moves from the atria in the ventricles. With hypertrophy, the left ventricle becomes thicker, tougher, denser - this leads to the fact that the process of relaxation, the stretching of the heart is hampered, becomes defective; Accordingly, the blood flow of such a ventricle is broken (decreases). Clinically, this phenomenon manifests itself short. The symptoms of diastolic heart failure in the form of shortness of shortness of breath and weakness for many years may be the only manifestation of GML. However, in the absence of adequate treatment of the main disease, symptoms will gradually increase, which will lead to a progressive decrease in exercise tolerance. The final of distant diastolic heart failure will be the development of systolic heart failure, the treatment of which is even more difficult. So, the GML is a direct path to heart failure, which means that the high risk of early cardiac death.

The following frequent complication of GML is the development of paroxysmal clear arrhythmia (atrial fibrillation). Violation Relaxation (diastole) of hypertrophied left ventricle inevitably entails an increase in blood pressure in it; This in turn makes the left atrium to decline stronger to "push" the required blood volume in the "Capacity" with increased pressure. However, the left atrium is a thin-walled cardiac chamber that cannot work for a long time in the supergrade; As a result, the left atrium is dilated (expanding) to accommodate excess blood. The dilatation of the left atrium is one of the most important risk factors for the development of atrial fibrillation. As a rule, the lesion of the left atrium is manifested only by atrial extrasystole; Subsequently, when the atrium "is sufficiently dilated" to "support" fibrillation, flickering arrhythmia occurs: first parole, then constant. Those risks that clarifying arrhythmias are in detail in detail are described in detail in a separate chapter.

Obstructive fainting. A rare variant of the flow of GML. It is almost always a complication of an asymmetric version of hypertrophic cardiomyopathy, when the thickness of the interventricular partition is so large that there is a threat of transient obstruction (overlapping) by blood flow in the region of the left ventricle. Paroxysmal obstruction (termination) of blood flow in this "critical place" will inevitably lead to a fainting. As a rule, the risk of obstruction development occurs when the thickness of the interventricular partition is exceeded 2 cm.

Stomatricular extrasystole - Another possible satellite with GML. It is known that any micro and macroscopic changes of the heart muscle can theoretically be complicated by the extrasystole. Hypertrophied myocardium is an ideal arrhythmogenic substrate. Clinical current ventricular extrasystole Against the background of GMLs, variabelno: more often, its role is limited to a "cosmetic arrhythmic defect". However, if the disease led to the GML is not treated (ignored), the restriction mode of intensive physical exertion is not observed, the development of life-degrading ventricular rhythm violations that launched by the extrasystole is possible.

Sudden heart death. The most severe complication of GML. Most often to such a final leads GMLG against the background of hypertrophic cardiomyopathy. Causes two. First, with this disease, the GML can be particularly massive, which makes myocardium extremely arrhythmogenic. Secondly, hypertrophic cardiomyopathy very often has an asymptomatic flow that does not allow patients to take preventive preventive measures In the form of restriction of intense physical exertion. Sudden heart death with other nosologies complicating GML, the phenomenon as a whole is rare, if only because the manifestation of these diseases begins with the symptoms of heart failure, which in itself makes the patient consult a doctor, which means there is a real opportunity to take the disease under control.

The possibility of regression GMLG. The probability of reducing the mass (thickness) of the myocardial of the left ventricle against the background of treatment depends on the cause of hypertrophy and its degree. A classic example is a sports heart, the walls of which are capable of decreasing to normal thickness after the cessation of a sports career.

The GML due to arterial hypertension or aortic stenosis can be successfully regressing with timely, full and long-term control over these diseases. However, it is true: only light hypertrophy is subject to absolute regression; In the treatment of hypertrophy of moderate, there is a chance to reduce it to easy; And heavy can "become medium". In other words than running processThe less chances to return everything completely to the original one. However, any degree of regression GML automatically means correctness in treating the main disease, which in itself reduces those risks that hypertrophy introduces subject to the subject.

With hypertrophic cardiomyopathy, any attempts to drug correction are meaningless. There are surgical approaches in the treatment of massive hypertrophy of the interventricular partition, which is complicated by the obstruction of the left ventricle's removal path.

The probability of regression GMLG against the background of obesity, in the elderly, with amyloidosis is practically absent.

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Version: MedElement disease reference

Obstructive hypertrophic cardiomyopathy (I42.1)

general information

Short description

Insulated hypertrophy of myocardials of unexplained tyro-du, the French Para Tolohanatet N. Lionville (1869) and L. Hallopeau (1869) were described in the second half of the 19th century (1869) and L. Hallopeau (1869). They mentioned the narrowing of the left ventricle's enduring path due to the clutch of the interventricular partition and gave this disease the name "left-sided muscular conus sandosus".

Hypertrophic cardiomyopathy- The disease of myocardium unknown etiology, inherited by an autosomal dominant path, characterized by the myocardial hypertrophy of the left and (or) occasionally of the right ventricle, more often, but not necessarily, asymmetric, as well as pronounced disorders of the diastolic filling of the left ventricle in the absence of dilatation of its cavity and the reasons for hypertrophy Hearts.

Classification

Currently adopted a hemodynamic classification of GKMP.

By the presence of a gradient systolic pressure in the left-handed cavity

Daughter
Obstructive form GKMP- The presence of a systolic pressure gradient in the left ventricular cavity.

Nonstructive form GKMP- Lack of systolic pressure gradient in the left ventricular cavity.

Hemodynamic version of obstructive GKPM
- with basal obstruction - subiutal obstruction alone.
- with labile obstruction - significant spontaneous oscillations of the intraventricular pressure gradient.
- with latent obstruction - obstruction occurs only when loading and provocative pharmacological samples.

According to the pressure gradient (when obstructive form)

1 stage - pressure gradient less than 25 mm RT st

2 Stage - less than 36 mm RT Art

3 Stage - less than 44 mm RT Art

4 Stage - from 45 mm RT Art

With the flow:
- Stable, benign flow.
- Sudden death.
- progressive course: strengthening shortness of shortness of breath, weakness, fatigue, pain syndrome (cardialgy, angina), synicopal and pressing states, etc.
- Development of flickering arrhythmia and related thromboembolic complications.
- "Finite Stage": the increase in heart failure phenomena due to the remodeling of the left ventricle and reduce its contractility.

The gradient in Volze is taken to measure with the help of Doppler-Ehoche, which eliminates the need for heart catheterization under GKMP (except for cases of suspected atherosclerotic damage to coronary arteries or heart valves).

Etiology and pathogenesis

GKMP is a hereditary disease that is transmitted as an autosomal dominant sign. The genetic defect occurs when mutation in one of 10 genes, each of which encodes the components of the Cardial Sarcomer protein and determines the development of myocardial hypertrophy. Currently, about 200 mutations responsible for developing the disease are identified.

Several pathogenetic disease development mechanisms are isolated:

- hypertrophy of the interventricular partition. As a result of the generic defect in the sarcomere of myocardium, disproportionate hypertrophy of the interventricular septum may develop, which in some cases occurs even in the period of embryonic morphogenesis. At the histological level, the changes in myocardial are characterized by the development of metabolic disorders in cardiomyocyte and a significant increase in the number of nucleoli in the cell, which leads to the arrangement of muscle fibers and development in the myocardium of connective tissue (English. Disarray phenomenon is the phenomenon of "disorder"). The disorganization of the heart muscle cells and the myocardial substitution by connecting tissue leads to a decrease in the pump function of the heart and serve as a primary arrhythmogenic substrate, which is predisposing to the occurrence of life-degraded tachyarithmia.
- obstruction of the weekend of the left ventricle. The obstruction of the Volga, which arises as a result of disproportionate hypertrophy of the interventricular septum as a result of disproportionate hypertrophy, which contributes to the contact of the front flap of the mitral valve with the interventricular partition and the sharp increase in the pressure gradient into the Volga during systole.
- Violation of the relaxation of myocardium left ventricle. The long-term existence of obstruction and hypertrophy of the interventricular septum leads to a deterioration in the active muscle relaxation, as well as to an increase in the rigidity of the LV walls, which causes the development of diastolic dysfunction of LV, and in the terminal phase of the disease - systolic dysfunction.
- myocardial ischemia.An important link of pathogenesis GKMP is the myocardium ischemia associated with the development of hypertrophy and diastolic dysfunction of LV, which leads to hypoperfusion and an increase in the array of myocardium. As a result, it is thinning the walls of the left ventricle, its remodeling and the development of systolic dysfunction.

Epidemiology

Hypertrophic cardiomyopathy is found with frequency1: 1000-1: 500. It is believed that it is most common among residents of Asian countries and the coast of the Pacific Ocean, especially in Japan. Men fall more often than women. More often occurs in people of young age, being frequent cause Sudden heart death. About half of all cases of the disease make up family forms. Annual mortality from GKMM is 1-6%.

Risk factors and groups

Risk factors of sudden death with hypertrophic cardiomyopathy:

Manifestation of the disease at the old age (up to 16 years),
- the presence in the family history of episodes of sudden death,
- frequent syncopal states,
- short-lighted episodes of ventricular tachycardia, identified with the 24-hour monitoring of the ECG,
- Pathological change in the level of blood pressure in the time of loads.

Clinical picture

Cymptoms, flow

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

Classic triad symptoms With hypertrophic cardiomyopy, it includes stress angina, shortness of breath with load and fainting. Painfulness In the chest, there are 75% of patients with hypertrophic cardiomyopathy, classical angina stainlessness - in 25%.

Dyspneaand often concomitant pain in the chest, dizziness, syncopal and pressing states are usually found with the stored systolic function of LV. The listed symptoms are associated with the occurrence of diastolic dysfunction of myocardium and other pathophysiological mechanisms (myocardial ischemia, the obstruction of the Volga and the concomitant mitral regurgitation, FP).

Chest pain In the absence of atherosclerotic damage to the coronary vessels, it can be used as typical for angina and atypical character.

Syncopal and dizziness It is primarily characteristic, first of all, for patients with obstructive form of GKMP due to hemodynamic obstruction (decrease in the lumen of Volge). In most cases, they arise suddenly against the background of complete health during the period of physical or emotional stress, however, they can occur alone. Most often, fainting is observed in painful young age, many of them have the episodes of ventricular tachycardia in many of them during the daily monitoring of ECGs, vision of conductivity.

In a significant number of patients (5-28%), atrial fibrillation arises that increases the risk of thromboembolic complications.

With obstructive form of hypertrophic cardiomyopathy, you are the following symptoms:

Systolic noise (Crescendo-diminuendo), which is not conducted or weakly carried out on carotid arteries and in the back area. The noise is caused by obstruction in the exile of blood from the left ventricle (WHO in the systole, when the hypertrophied interventricular neglence and the front sash of the mitral valve move on to meet each other);

The noise is enhanced by decreasing the filling of the heart and the reduced of the total peripheral vascular resistance (ridges from the position of sitting in squatting, straining, taking the nit-roglisserin) and weakens with increasing heart filling, increasing the total peripheral vascular resistance (in the lying position, sitting on squatting, when compressing fists);

Pulsation sleepy arteries, the rapid "ripple" pulse at palpation of sleepy arteries, which are a reflection of a very fast exile of blood in the first half of systole;

Stressed long-lasting top push, occupying the whole systole up to the second tone, which is a sign of the hypertro-fition of the left ventricle;

When palpation of the top shock in the position on the left side with the breathing delay on the exhalation, it is sometimes felt by the double lifting.

With auscultation, the tones of the heart is deaf, Ivton is detected.

Diagnostics

ECG in 12 leads.

Different changes in ECG are registered in 92-97% of patients, referred to the earliest manifestation of GKMP and may precede the development of myocardial hypertrophy, detected by ECCG. Strictly specific ECG signs of GKMM, as well as clinical, does not exist.
Most often there are changes in the ST segment, the inversion of the teeth T, the signs of more or less pronounced hypertrophy of the left ventricle, the deep teeth Q and the signs of hypertrophy and the overload of the left atrium. The blockade of the reserved branch of the left leg of the beam of His and the signs of hypertrophy of the right atrium are noted, in isolated cases, the right ventricle. The full blockade of the feet of the Gis beam is not characteristic. Common ECG changes under GKMP are negative teeth, in some cases, in combination with the depression of ST segment, which are registered in 61-81% of patients. Giant, in excess of 10 mm, negative teeth of T in breasts are very characteristic of the top-like form of this disease, at which they have an important diagnostic value. Changes in the final part of the ventricular complex under GKMM are caused by myocardial ischemia or finelyocal cardiosclerosis. Detection of deep q and negative tons, especially with complaints of anginal pain, serves as a frequent cause of erroneous diagnosis of IBS and causes the need to carry out the differential diagnosis of GKMM with this disease.

Halter monitoring ECG. Conducting Holter monitoring ECG to diagnose rhythm and conductivity disorders shown by a high risk of sudden death, primarily with syncopal states, the presence of cases of sudden death in the family, as well as with clinical and ECG signs of myocardial ischemia. It is also advisable to use it also to control the effectiveness of antiarrhythmic therapy.

Phonocardiography. It is very characteristic, but non-specific, is a pathological strengthening of III and especially the IV heart tones. The factory sign of subaartal obstruction is the so-called late, not associated with the I tone, systolic noise of rhomboid or tanning form with the epicenter at the top or in the III-IV intercostal Breasts. It is carried out in the axillary region and less often on the base of the heart and the vessels of the neck. Distinctive features Noise, allowing to suspect obstructive GKMP, serve specific changes in its amplitude and duration in physiological and pharmacological samples aimed at increasing or decreasing the degree of obstruction and associated mitral insufficiency. A similar nature of the noise dynamics has not only diagnostic value, but also is a valuable criterion for the differential diagnosis of GKMP with primary lesions of mitral and aortic valves. The noise can be preceded by an additional tone, formed by contacting the mitral sash with the interventricular partition. The part of the patients in diastole is recorded next for the third-tone short low amplitude noise of the tributary, that is, relative mitral or occasionally of the trickspeed stenosis. In the latter case, the noise is enhanced by inhale. With a significant severity of the obstacle, blood flow is determined by the paradoxical splitting of the second tone due to the elongation of the period of expulsion of the left ventricle in proportion to the size of the systolic gradient of the pressure.

Radiographic examination of the chest.Data x-ray research Hearts are little informative. Even with significant myocardial hypertrophy, significant changes in the shadow of the heart may be absent, since the volume of the left ventricle cavity is not changed or reduced. Part of patients has a slight increase in the left ventricular arcs and the left atrium and the rounding of the top of the heart, as well as the signs of moderate venous pulmonary hypertension. Aorta is usually reduced.

Dopplerhocardiography
None of Echocrous signs of GKMM, despite the high sensitivity, is not pathognomonic.

Basic echo-signs :
- Asymmetric left ventricular myocardial hypertrophybut. The generally accepted criterion of GKMP - the thickness of the interventricular partition is more than 15 mm with a normal or increased thickness of the rear wall of the LV. Given that the disease is genetically determined, the degree of hypertrophy may be different. However, the presence of symmetric hypertrophy does not exclude the diagnosis of GKMP.

- obstruction of the left ventricle. The hemodynamic systolic gradient of pressure in Volge is determined using Doppler scanning. The gradient of more than 30 mm Hg is considered diagnostically significant. (flow rate in Volge - 2.7 m / s). Perform a sample with exercise to determine the degree of gradient in Volge. Sample with dobutamine is not used due to the high risk of developing rhythm violations.
- The overall movement of the front flap of the mitral valve. Also often detect the dilatation of the left atrium, mitral regurgitation, and in the terminal stage - and dilatation of LV.

Stress-Ehocheg It is used to identify the coronary heart disease concomitant GKMP, which has an important prognostic and therapeutic value.

Radionuclide valrenculography As the most reproducible method for estimating systolic and diastolic function, not only the left, but also right ventricle is used mainly to monitor the patients with GKMP in dynamics and to assess the effectiveness of therapeutic activities.

Magnetic resonance tomography withit is the most accurate method for assessing the morphology of the heart, which plays a key role for the diagnosis of GKMM. Thus, magnetic resonance imaging allows you to get additional information about the distribution of hypertrophy in 20-31% of patients with GCMP (F.Sardinelli et al., 1993; J.Posma et al., 1996) and ensures the measurement of thickness 97% of the segments Left ventricle compared to 67% when applying EchoCG (G.pons-Llado et al., 1997). In a way, magnetic resonance tomography can serve as a kind of "gold standard" to assess the prevalence and severity of myocardial hypertrophy in patients with GKMP.

Positron emission tomography Represents a unique opportunity for non-invasive valuation of regional perfusion and myocardial metabolism. The preliminary results of its use under GKMM showed a decrease in the coronary expansion reserve not only in hypertrophied, but also unchanged in the thickness of the left ventricle segments, which is especially expressed in patients with anginal pain. Violation of 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 left ventricular path of the left ventricle alone or during provocative tests. This feature is characteristic of obstructive GKMM and is not observed in the unstructive form of the disease, which does not allow to exclude GKMM in its absence. When registering the pressure gradient in the left ventricle cavity in relation to its enduring path, it is necessary to make sure that it is due to the subaartal obstruction of the blood is due, and is not a consequence of the dense grapping of the end of the catheter with the walls of the ventricle with the so-called "elimination" or "obliteration" of its cavity. Along with the subaartal gradient, an important sign of the obstacle to the expulsion of blood from the left ventricle is the change in the form of the pressure curve in the aorta. As well as on the sphygmogram, it acquires the form of "peak and domes". It is a significant part of patients with GKMM, regardless of the presence or absence of a subaartal gradient, an increase in finite-diastolic pressure in the left ventricle and pressure on the paths of its inflow is determined - in the left atrium, pulmonary veins , Long capillaries and pulmonary artery. Wherein pulmonary hypertension Wears the character of passive, venous. An increase in the finite-diastolic pressure in the hypertrophied left ventricle is due to a violation of its diastolic adhesiveness characteristic of GKMP. Sometimes, in the terminal stage of the development of the disease, it is aggravated by the attachment of the systolic dysfunction of myocardium.

Coronary angiography. It is performed under GKMP and constant stunned pains (frequent attacks of angina):

People over 40 years old;
in individuals with the risk factors IBS;
In persons with an established diagnosis of CHD to invasive intervention (for example, septal mioectomy or alcoholic septal ablation).

Endomocardial biopsyleft or right ventricles are recommended in cases where there are doubts about the diagnosis after the clinical and instrumental examination. When identifying the characteristic pathological characteristics of the disease, a conclusion of morphological changes in myocardium is a clinical diagnosis of GKMP. With the other side, the detection of structural changes specific for any other myocardial lesion (for example, amyloidosis) makes it possible to eliminate GKMP.

In the presence of DopplerHocardiography and magnetic resonance tomography, EMB for the diagnosis of GKMP is now practically not used.

Laboratory diagnostics

In order to eliminate other most common cardiological diseases, it is necessary to carry out a biochemical blood test (lipid spectrum, myocardial necrosis biomarkers, blood serum glucose), evaluation of the functional state of the kidneys, liver and general blood tests and urine.

Differential diagnosis

Differential diagnosis is carried out with a number of diseases accompanied by the development of left ventricular hypertrophy, primarily the "heart of an athlete", acquired and congenital defects, DCMP, and with a tendency to increase blood pressure - essential arterial hypertension. Differential diagnosis with heart defects, accompanied by systolic noise, is particularly important in cases of obstructive form of GKMP. In patients with focal and ischemic changes on ECG and angiositic pain, the primary task is the differential diagnosis with IBS. When prevailing in the clinical picture of signs of congestive heart failure in combination with a relatively small increase in the size of the heart GKMP should be differentiated from the atrial mysteries, chronic pulmonary heart and diseases occurring with restriction syndrome - constrictive pericarditis, amyloidosis, hemochromatosis and sarcoidosis of the heart and restrictive cardiomyopathy.

Coronary artery disease. Most often, the GKMM has to differentiate with chronic and less as sharp forms of IBS. In both cases, anginal pain in the field of heart, shortness of breath, heart rate disorders, concomitant arterial hypertension, additional tones in diastole, finely and large-scale changes and signs of ischemia to the ECG value for the diagnosis has echoca, in which part of patients has The intrinsic segmentation disorders, moderate dilatation of the left ventricle and the reduction of its emission fraction are determined. Left ventricular hypertrophy is very moderate and more often is symmetrical. The impression of the disproportionate thickening of the interventricular partition can create the presence of akinesia zones due to post-infarction cardiosclerosis in the area of \u200b\u200bthe rear wall of the left ventricle with compensatory myocardial hypertrophy. At the same time, as opposed to asymmetric hypertrophy of the interventricular partition as a form of GKMP, the hypertrophy of the partition is accompanied by hyperkinesia. In cases of noticeable dilatation of the left atrium, due to the concomitant mitral regurgitation, the Dilatation of the left ventricle is invariably notable, the dilatation of the left ventricle is unchanged. Confirm the diagnosis of GKMM allows the detection of signs of a subaartal pressure gradient. In the absence of echochegous data in favor of subaartal obstruction, differential diagnosis is significantly hampered. The only reliable method of recognition or exclusion of the IBS in such cases is X-ray-contrast coronorography. In persons of middle and older, especially in men, it is necessary to keep in mind the possibility of a combination of GKMP with IBS.

Essential arterial hypertension. For differential diagnosis, the greatest complexity is the GKMP, which flows with an increase in blood pressure, which should be distinguished from the isolated essential arterial hypertension, accompanied by the left ventricle hypertrophy with the disproportionate thickening of the interventricular partition. In favor of essential arterial hypertension, a significant and persistent increase in blood pressure is evidenced, the presence of retinopathy, as well as an increase in the thickness of intima and the media of sleepy arteries, not characteristic of patients with GKMP. Special attention should be paid to identifying signs of subaartal obstruction. In the absence of a subaartal pressure gradient on a probable GPM, in contrast to essential arterial hypertension, a significant severity of asymmetric hypertrophy of the interventricular septum is indicated with an increase in its thickness by more than 2 times compared to the rear wall of the left ventricle, as well as the detection of GKMM at least one of the 5 adult blood relatives. On the contrary, in the absence of signs of GKMP in 5 and more family members, the probability of this disease does not exceed 3%.

With a combination of left ventricular hypertrophy with systolic noise, it is necessary to carry out a differential diagnosis of obstructive GPM with heart defects, primarily the insufficiency of the mitral valve, the valve and was aorticed by the membrane stenosis of the aorta, coarctation of the aorta and the defect of the interventricular partition. In this case, the nature of the dynamics of the reverse current of blood than auscultation, FKG and DopplerHocardiography, under the influence of the change in the preload and post-loading of the left ventricle using the change of body position, the sample of the waltzalver and the introduction of vasopressor and vasodilator drugs.

Unlike GPM, when rheumatic mitral insufficiency The volume of regurgitation into the left atrium increases with increasing blood pressure, that is, the obstacles to exile, and decreases with a decrease in the venous inflow in the standing position or after inhalation of the amylnitrite. In favor of the diagnosis of GKMP, family history, the presence of anginal pain, focal and ischemic changes to the ECG. Confirm the diagnosis allows the detection of signs of subaartal obstruction during dopplerhocardiography.

Certain difficulties may occur with the differential diagnosis of GKMP and matral valve prolapse. In both diseases, there is a tendency to heartbeat, interruptions, dizziness and fainting, "late" systolic sound over the top of the heart and the same nature of its dynamics under the influence of physiological and pharmacological samples. At the same time, the mitral valve prolapse, in contrast to GKMP, is characterized by a smaller severity of left ventricular hypertrophy and the lack of focal changes to the ECG. The final diagnosis can be made on the basis of DopplerHocardiography data, including percussive.

Valve stenosis of the aorta. In terms of cases, the epicenter of the systolic noise of the valve stenosis of the mouth of the aorta is determined at the point of Botkin and above the top of the heart, which can resemble an auscultative picture of obstructive GKMP. For both diseases, anginal pain, shortness of breath, syncope, signs of Hypertrophy of the left ventricle, changes of the ST and Tusque T to ECG, as well as an increase in the thickness of the myocardial of the left ventricle at a constant or reduced sizes of its cavity at echoC and AKG, is equally characterized. The stenosis of the mouth of the aorta helps the definition of the characteristics of the pulse, conducting systolic noise on the neck vessels, the presence of post -tenotic expansion of the ascending aorta and signs of fibrosis or calcinosis of the aortic valve during radiography and echoch, as well as changes in the sphygmogram in the form of a "cock crest". Confirm the diagnosis of the stenosis of the aorta mouth allows the detection of the systolic pressure gradient at the valve level during dopplerhocardiography and catheterization of the heart.

A more difficult task is the differential diagnosis of obstructive GKMP and Membranous subautal stenosis. In favor of GKMM, a family history, a characteristic form of a spigmogram curve and a later occurrence of the systolic cover of aortic valve at ECCG), while on the likely membrane stenosis of the aorta's mouth indicates the concomitant aortic regurgitation - the frequent complication of this congenital vice. Dopplerhocardiography and invasive examination, allowing to determine the localization and nature (fixed or dynamic) obstacle to the left ventricle to be determined.

Patient Catching aorta, like GKMP, characteristic of shortness of breath, dizziness and cardialgia, arising in young age and combined with systolic noise in the precordial region and signs of left ventricular hypertrophy at ECG and Ehocheg. Recognition of these diseases usually does not cause difficulties and possibly already at the stage of clinical examination when pathogenous to coarctation aorta increases upper limbs And its decline on the lower. In doubtful cases, it is possible to confirm the diagnosis of congenital heart defects allow the data of magnetic resonance imaging and X-ray-contrast ahrortography.

Defect of the interventricular partition.In asymptomatic patients of young age with coarse systolic noise in the III-IV, the inter estreon at the left edge of the sternum and signs of the left ventricle hypertrophy has to carry out a differential diagnosis of obstructive GKMP with a defect of the interventricular partition. The distinguishing features of this congenital defect with a non-invasive examination are "heart hump" and systolic jitter in the place of listening to noise, its connection with I tone, as well as a noticeable increase in the pulmonary artery arc on radiographs of the heart. The final diagnosis can be delivered by Doppler Ehoche, and in particularly complex cases - invasive heart examination.

Complications

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

- Sudden heart death (WSS)
- thromboembolia
- progression of chronic heart failure (CHHN).

Treatment

TO general events The limitation of significant physical exertion and prohibition of sports that can cause aggravation of myocardial hypertrophy, increasing the intraventricular gradient of pressure and risk of Sun. To prevent infectious endocarditis in situations associated with the development of bacteremia, antibiotic-porphylaxis is recommended for obstructive formas, similar to those in patients with heart defects.

The basis of drug therapy GKMM is preparations with a negative inotropic effect: β-adrenoblays and calcium channel blockers (verapamil).

β-adrenoblocators became the first and remain to this day most effective group medicines used in the treatment of GKMM. They should be prescribed to patients regardless of the severity of the gradient of the intragericular pressure alone. It is preferable to refrain from the purpose of the β-aope-noretoletor blockers with internal sympathomimetic activity (PIN-dol, oxprenolol). Propranolol is prescribed at a dose of 240-320 mg per day and more (maximum daily dose - 480 mg), metoprolol - at a dose of 200 mg per day and more. Cardooselective Beta-Adrenorezp-Torov blockers with hypertrophic cardiomyopathy do not have advantages over non-selective, since in high doses the selectivity is practiced.

In the case of contraindications to the purpose of beta-adrenoreceptor blockers or the incomplete disappearance of symptoms, alternative may be calcium channel blockers. Among the calcium channel blockers with the drug selection is verapamil (isoptin, fooptin). It provides a symptomatic effect in 65-80% of patients. When appropriate, calcium channels are prescribed maximum caution in the presence of pronounced hypertrophy and very high pressure on-completely left ventricle. It should be borne in mind that the blockers of calcium channels, including verapamil, with long-term use of the Mo-Gut to increase the diastolic pressure and reduce the cardiac output. Treatment with verapamil should be started from appointment at low doses - at 20-40 mg 3 times a day, gradually increasing to a daily dose of 240-320 mg and more. Clinical improvement in the reception of verapamil is approached by increasing tolerance to physical exertion.

For the treatment of arrhythmias used antiarrhythmic drugs from which the most effective are dizeciramide and amiodar. Dizeciramide (rhythmile) relating to the antiarrhythmics of IA class has a pronounced negative inotropic effect, in patients with GKMP, it is capable of reduced the level of obstruction of the output tract of the left ventricle, has a positive effect on the diastole structure. The initial dose is usually 400 mg / day with a gradual increase of up to 800 mg. At the same time, it is necessary to control the duration of the Q-T interval on the ECG.
The only drug, against which today, the elimination of ventricular tachyrhythmia is noted, the decline in the incidence of sudden death and the improvement of the disease forecast is amiodar. Amiodarons are permanent in a daily dose of 1200 mg for 5-7 days, then in a daily dose of 800 mg and 600 mg for the 2nd and 3rd week of treatment, followed by a transition to a supporting daose dose of 200 mg.

In the treatment of GKMP with symptoms of heart failure, the following points must be taken into account:
contraindicated diuretics, Which, although the effects, tigalously reduce the stagnation in the lungs, however, can cause hypovolemia that can enhance the obstruction of the enduring path in patients! Too long-term use of diuretics can determine the reduction of shock volume and cardiac output.
Vasodilators (nitroglycerin, sodium nitroprusside) have limited use due to the possible risk of developing pronounced hypotension and reduce the size of the left ventricular polo, which may worsen the patient's condition.
Inotropic agents aimed at stimulating systolic ejection (heart glycosides and pressor amines), They may produce a non-favorable hemodynamic effect - they enhance the obstruction of the ending path and do not reduce the increased finite-diastolic pressure, can cause the development of asistolia. Nevertheless, digoxin can be used in patients with diastolic dysfunction and atrial fibrillation to reduce heart rate and / or to restore sinus rhythm.
Drug selection for CXN treatment may be Inhibi Torah ACE Because of their ability to block the renin-angiotensin system and cause the opposite development of the left ventricular hypertrophy.

In the absence of a clinical effect of active drug therapy, symptomatic patients with III-IV functional class according to the classification of the New York Association of Cardiologists with a pronounced asymmetric hypertrophy of the MZHP and a subaartal pressure gradient at rest equal to 50 mm Hg. Art. And more, shown surgery.
Classical technique - Crystal septal meshtomy proposed by AgMorrow. The operation provides a good symptomatic effect with a complete elimination or a significant decrease in the intraventricular pressure gradient in 95% of patients and a significant decrease in finite-diastolic pressure in LV 66% of patients.
In some cases, if there are additional indications for reducing the severity of obstruction and mitral regurgitation, the valveloplasty or prosthetics of the mitral valve with a low-profile prosthesis is simultaneously performed.

In recent years, growing interest is the study of the possibility of using as an alternative to surgical treatment of patients with obstructive GKMP sequential two-chamber electrocardialization with a shortened atrioventricular delay.Thus, the change in the sequence of propagation of the excitation wave and reduction of ventricles, which embraces the top, and then the MZHP, leads to a decrease in the subportal gradient due to a decrease in the regional coherence of the MZHP and, as a result, expand the extension tract of LV.

Another alternative treatment for refractory obstructive GKMP is transcathetter Alcohol Septal Ablation The technique implies infusion through a balloon catheter into a perforant septal branch of 1-3 ml of 95% of alcohol, as a result of which an infarction of the hypertrophied MZhP department, exciting from 3 to 10% of the mass of LV myocardial (up to 20% of the mass MZP). This leads to a meaningful decrease in the severity of the obstruction of the weekend and mitral insufficiency, objective and subjective symptoms of the disease, at the same time, in 5-10% of cases, it is necessary to implant the permanent exemption in connection with the development of the atrioventricular blockade of a high degree. In addition, it has now been not proven to the positive impact of the transcatethette ablation on the forecast, and the operating mortality rate (1-2%) is not different from that during the operation of septal mestectomy, which is considered to date the "golden standard" of the treatment of patients with GKMP with severe symptoms and obstruction Output tract of LV, resistant to pharmacotherapy.

Forecast

The forecast is most favorable with a long-term asymptomatic course of the disease and an unbounded family history, especially at the top form of GKMP. In some such cases, the disease may not be reflected on life expectancy.

Most patients with GKMM die suddenly, regardless of the possibility of the disease. An unfavorable prognosis in children, most of which are asymptomatic, is associated with a burded family history regarding sudden death. Adolescents and young and middle-aged people (from 15 to 56 years old) are the main factor that aggresses the forecast is the exposure to fainting. Patients in older people are prognostically unfavorable are shortness of breath and pain in the heart area during exercise.

5 main options for the course of the disease and outcomes are distinguished:
- stable, benign flow;
- sudden death;
- progressive flow - strengthening of shortness of shortness of breath, weakness, fatigue, pain syndrome (atypical pain, angina), the appearance of synicopal states, disorders of LV systolic dysfunction;
- "final stage" - further progression of phenomena of stagnant heart failure associated with remodeling and LV systolic dysfunction;
- Development of atrial fibrillation and complicated complications associated with it, in particular thrombometric.

Prevention

The prevention of the disease is to identify it in the early stages, which allows you to start early treatment of the disease and prevent the development of expressed myocardial hypertrophy. EchoCG should be made in obligatory in the "blood" (genetic) relatives of the patient. All other persons showed a detailed examination in the presence of similarity of manifestations: fainting, angina, etc. Screening (all in a row) ECG and ECOGG during annual dispensarization are also useful. In patients with obstructive form GKMP, it is also necessary to carry out the prevention of infectious endocarditis (antibioticoprophylaxis, etc.), since the presence of obstruction creates conditions for the development of this dangerous disease.

Information

Information

1. Treatment of heart failure. Recommendations of the Working Group on the Study of the Heart Failure of the European Society of Cardiologists. Rus. honey. journal Application. 1999.
2. Amosova E.N. Cardiomyopathy. Kiev: "Book Plus", 1999; 421 p.
3. Kushakovsky M.S. Chronic stagnant heart failure. Idiopathic myocardiopathy. St. Petersburg: "Folitan", 1998; 320 p.
4. Moiseev V.S., Sumarokov A.V., Shzyazhkin V.Yu. Cardiomyopathy. M.: Medicine 1993; 176 p.
5. Guide on Ambulatory -Poliklinic Cardiology under the Red Belenkova Yu.N, Oganova R.G., Publishing House Gootar Media, - 2007.-400 p.
6. Polyakov V.P., Nikolaevsky V.N., Picko G.A. Non-cultural and infectious diseases of the heart disease (modern aspects of clinic, diagnosis, treatment): monograph. - Samara, 2010, -355С.
7. Internal illnesses By Tinsley R. Harrison / Ed. E. Faucci, Yu. Brownwald, K. Isselbahher, J. Wilson, J. Martina, D. Kasper, S. Khauzer and D.Ligo: 7T. - M.: Practice - Mak-Grow_hill, 2005.
8. Clinical recommendations. Cardiology / Ed. Yu.N. Belenkova, R.G. Oganova. - M.: Gootar Media, 2007.
9. B.Griffin, E.Topol "Cardiology" Moscow, 2008
10. Kovalenko V.N., Nesukai E. G. Non-cultural heart disease: practical guide / ed. V. N. Kovalenko. K.: "Morion", 2001. - 480 p.

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Version: MedElement disease reference

Other hypertrophic cardiomyopathy (I42.2)

general information

Short description

Nonstructive hypertrophic cardiomyopathy

Insulated hypertrophy of myocardials of unexplained tyro-du, the French Para Tolohanatet N. Lionville (1869) and L. Hallopeau (1869) were described in the second half of the 19th century (1869) and L. Hallopeau (1869). They mentioned the narrowing of the left ventricle's enduring path due to the clutch of the interventricular partition and gave this disease the name "left-sided muscular conus sandosus".

Hypertrophic cardiomyopathy- The disease of myocardium unknown etiology, inherited by an autosomal dominant path, characterized by the myocardial hypertrophy of the left and (or) occasionally of the right ventricle, more often, but not necessarily, asymmetric, as well as pronounced disorders of the diastolic filling of the left ventricle in the absence of dilatation of its cavity and the reasons for hypertrophy Hearts.

Classification

Classification of cardiomyopathy

Localization

1. Hypertrophy LJ.

a) asymmetric:

Hypertrophy of basal departments MZP

Total hypertrophy mzhp.

Total hypertrophy of MZHP and free wall of lz

Hypertrophy of the top of the heart with a possible propagation on the free wall of the LV and the partition

b) symmetric (concentric)

2. Hypertrophy of PJ

I. I. Hemodynamic form

1. UNCTATIVE

2. Obstructive

III. According to the pressure gradient (when obstructive form)

1 stage - pressure gradient less than 25 mm RT st

2 Stage - less than 36 mm RT Art

3 Stage - less than 44 mm RT Art

4 Stage - from 45 mm RT Art

IV . On clinical manifestations

Asymptomic flow

Syncopal states

Violations of rhythm

V. In clinical flow stages

1. Compensation

2. Subcompensation

Etiology and pathogenesis

GKMP is a hereditary disease that is transmitted as an autosomal dominant sign. The genetic defect occurs when mutation in one of 10 genes, each of which encodes the components of the Cardial Sarcomer protein and determines the development of myocardial hypertrophy. Currently, about 200 mutations responsible for developing the disease are identified.

Several pathogenetic disease development mechanisms are isolated:

- hypertrophy of the interventricular partition. As a result of the generic defect in the sarcomere of myocardium, disproportionate hypertrophy of the interventricular septum may develop, which in some cases occurs even in the period of embryonic morphogenesis. At the histological level, the changes in myocardial are characterized by the development of metabolic disorders in cardiomyocyte and a significant increase in the number of nucleoli in the cell, which leads to the arrangement of muscle fibers and development in the myocardium of connective tissue (English. Disarray phenomenon is the phenomenon of "disorder"). The disorganization of the heart muscle cells and the myocardial substitution by connecting tissue leads to a decrease in the pump function of the heart and serve as a primary arrhythmogenic substrate, which is predisposing to the occurrence of life-degraded tachyarithmia.
- obstruction of the weekend of the left ventricle. The obstruction of the Volga, which arises as a result of disproportionate hypertrophy of the interventricular septum as a result of disproportionate hypertrophy, which contributes to the contact of the front flap of the mitral valve with the interventricular partition and the sharp increase in the pressure gradient into the Volga during systole.
- Violation of the relaxation of myocardium left ventricle. The long-term existence of obstruction and hypertrophy of the interventricular septum leads to a deterioration in the active muscle relaxation, as well as to an increase in the rigidity of the LV walls, which causes the development of diastolic dysfunction of LV, and in the terminal phase of the disease - systolic dysfunction.
- myocardial ischemia.An important link of pathogenesis GKMP is the myocardium ischemia associated with the development of hypertrophy and diastolic dysfunction of LV, which leads to hypoperfusion and an increase in the array of myocardium. As a result, it is thinning the walls of the left ventricle, its remodeling and the development of systolic dysfunction.

Epidemiology

Hypertrophic cardiomyopathy is found with frequency1: 1000-1: 500. It is believed that it is most common among residents of Asian countries and the coast of the Pacific Ocean, especially in Japan. Men fall more often than women. More often occurs in people of young age, being a frequent cause of sudden cardiac death. About half of all cases of the disease make up family forms. Annual mortality from GKMM is 1-6%.

Risk factors and groups

Risk factors of sudden death with hypertrophic cardiomyopathy:

Manifestation of the disease at the old age (up to 16 years),
- the presence in the family history of episodes of sudden death,
- frequent syncopal states,
- short-lighted episodes of ventricular tachycardia, identified with the 24-hour monitoring of the ECG,
- Pathological change in the level of blood pressure in the time of loads.

Clinical picture

Cymptoms, flow

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

Classic triad symptoms With hypertrophic cardiomyopy, it includes Stress angina, shortness of breath with load and fainting. Painting sensations in the chest are noted in 75% of patients with hypertrophic cardiomyopathy, classical angina stainlessness - in 25%.

Dyspnea and often concomitant pain in the chest, dizziness, syncopal and pressing states are usually found with the stored systolic function of LV. The listed symptoms are associated with the occurrence of diastolic dysfunction of myocardium and other pathophysiological mechanisms (myocardial ischemia, the obstruction of the Volga and the concomitant mitral regurgitation, FP).

Chest pain In the absence of atherosclerotic damage to the coronary vessels, it can be used as typical for angina and atypical character.

Syncopal and dizziness It is primarily characteristic, first of all, for patients with obstructive form of GKMP due to hemodynamic obstruction (decrease in the lumen of Volge). In most cases, they arise suddenly against the background of complete health during the period of physical or emotional stress, however, they can occur alone. Most often, fainting is observed in painful young age, many of them have the episodes of ventricular tachycardia in many of them during the daily monitoring of ECGs, vision of conductivity.

In a significant number of patients (5-28%), atrial fibrillation arises that increases the risk of thromboembolic complications.

With an objective study of patients with a unstructive form of hypertrophic cardiomyopathy, the deviations from the norm may not be, but sometimes the increase in the duration of the top shock, the Ivton of the heart.

Diagnostics

ECG in 12 leads.

Different changes in ECG are registered in 92-97% of patients, referred to the earliest manifestation of GKMP and may precede the development of myocardial hypertrophy, detected by ECCG. Strictly specific ECG signs of GKMM, as well as clinical, does not exist.
Most often there are changes in the ST segment, the inversion of the teeth T, the signs of more or less pronounced hypertrophy of the left ventricle, the deep teeth Q and the signs of hypertrophy and the overload of the left atrium. The blockade of the reserved branch of the left leg of the beam of His and the signs of hypertrophy of the right atrium are noted, in isolated cases, the right ventricle. The full blockade of the feet of the Gis beam is not characteristic. Common ECG changes under GKMP are negative teeth, in some cases, in combination with the depression of ST segment, which are registered in 61-81% of patients. Giant, in excess of 10 mm, negative teeth of T in breasts are very characteristic of the top-like form of this disease, at which they have an important diagnostic value. Changes in the final part of the ventricular complex under GKMM are caused by myocardial ischemia or finelyocal cardiosclerosis. Detection of deep q and negative tons, especially with complaints of anginal pain, serves as a frequent cause of erroneous diagnosis of IBS and causes the need to carry out the differential diagnosis of GKMM with this disease.

Halter monitoring ECG. Conducting Holter monitoring ECG to diagnose rhythm and conductivity disorders shown by a high risk of sudden death, primarily with syncopal states, the presence of cases of sudden death in the family, as well as with clinical and ECG signs of myocardial ischemia. It is also advisable to use it also to control the effectiveness of antiarrhythmic therapy.

Phonocardiography. It is very characteristic, but non-specific, is a pathological strengthening of III and especially the IV heart tones. The factory sign of subaartal obstruction is the so-called late, not associated with the I tone, systolic noise of rhomboid or tanning form with the epicenter at the top or in the III-IV intercostal Breasts. It is carried out in the axillary region and less often on the base of the heart and the vessels of the neck. The distinguishing features of noise, allowing to suspect obstructive GKMP, serve specific changes in its amplitude and duration in physiological and pharmacological samples aimed at increasing or decreasing the degree of obstruction and associated mitral insufficiency. A similar nature of the noise dynamics has not only diagnostic value, but also is a valuable criterion for the differential diagnosis of GKMP with primary lesions of mitral and aortic valves. The noise can be preceded by an additional tone, formed by contacting the mitral sash with the interventricular partition. The part of the patients in diastole is recorded next for the third-tone short low amplitude noise of the tributary, that is, relative mitral or occasionally of the trickspeed stenosis. In the latter case, the noise is enhanced by inhale. With a significant severity of the obstacle, blood flow is determined by the paradoxical splitting of the second tone due to the elongation of the period of expulsion of the left ventricle in proportion to the size of the systolic gradient of the pressure.

Radiographic examination of the chest.The radiological research data is little informative. Even with significant myocardial hypertrophy, significant changes in the shadow of the heart may be absent, since the volume of the left ventricle cavity is not changed or reduced. Part of patients has a slight increase in the left ventricular arcs and the left atrium and the rounding of the top of the heart, as well as the signs of moderate venous pulmonary hypertension. Aorta is usually reduced.

Dopplerhocardiography
None of Echocrous signs of GKMM, despite the high sensitivity, is not pathognomonic.

Basic echo-signs :
- Asymmetric left ventricular myocardial hypertrophybut. The generally accepted criterion of GKMP - the thickness of the interventricular partition is more than 15 mm with a normal or increased thickness of the rear wall of the LV. Given that the disease is genetically determined, the degree of hypertrophy may be different. However, the presence of symmetric hypertrophy does not exclude the diagnosis of GKMP.

- obstruction of the left ventricle. The hemodynamic systolic gradient of pressure in Volge is determined using Doppler scanning. The gradient of more than 30 mm Hg is considered diagnostically significant. (flow rate in Volge - 2.7 m / s). Perform a sample with exercise to determine the degree of gradient in Volge. Sample with dobutamine is not used due to the high risk of developing rhythm violations.
- The overall movement of the front flap of the mitral valve. Also often detect the dilatation of the left atrium, mitral regurgitation, and in the terminal stage - and dilatation of LV.

Stress-Ehocheg It is used to identify the coronary heart disease concomitant GKMP, which has an important prognostic and therapeutic value.

Radionuclide valrenculography As the most reproducible method for estimating systolic and diastolic function, not only the left, but also right ventricle is used mainly to monitor the patients with GKMP in dynamics and to assess the effectiveness of therapeutic activities.

Magnetic resonance tomography withit is the most accurate method for assessing the morphology of the heart, which plays a key role for the diagnosis of GKMM. Thus, magnetic resonance imaging allows you to get additional information about the distribution of hypertrophy in 20-31% of patients with GCMP (F.Sardinelli et al., 1993; J.Posma et al., 1996) and ensures the measurement of thickness 97% of the segments Left ventricle compared to 67% when applying EchoCG (G.pons-Llado et al., 1997). In a way, magnetic resonance tomography can serve as a kind of "gold standard" to assess the prevalence and severity of myocardial hypertrophy in patients with GKMP.

Positron emission tomography Represents a unique opportunity for non-invasive valuation of regional perfusion and myocardial metabolism. The preliminary results of its use under GKMM showed a decrease in the coronary expansion reserve not only in hypertrophied, but also unchanged in the thickness of the left ventricle segments, which is especially expressed in patients with anginal pain. Violation of 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 left ventricular path of the left ventricle alone or during provocative tests. This feature is characteristic of obstructive GKMM and is not observed in the unstructive form of the disease, which does not allow to exclude GKMM in its absence. When registering the pressure gradient in the left ventricle cavity in relation to its enduring path, it is necessary to make sure that it is due to the subaartal obstruction of the blood is due, and is not a consequence of the dense grapping of the end of the catheter with the walls of the ventricle with the so-called "elimination" or "obliteration" of its cavity. Along with the subaartal gradient, an important sign of the obstacle to the expulsion of blood from the left ventricle is the change in the form of the pressure curve in the aorta. As well as on the sphygmogram, it acquires the form of "peak and domes". It is a significant part of patients with GKMM, regardless of the presence or absence of a subaartal gradient, an increase in finite-diastolic pressure in the left ventricle and pressure on the paths of its inflow is determined - in the left atrium, pulmonary veins , Long capillaries and pulmonary artery. In this case, the pulmonary hypertension is characteristic of passive, venous. An increase in the finite-diastolic pressure in the hypertrophied left ventricle is due to a violation of its diastolic adhesiveness characteristic of GKMP. Sometimes, in the terminal stage of the development of the disease, it is aggravated by the attachment of the systolic dysfunction of myocardium.

Coronary angiography. It is performed under GKMP and constant stunned pains (frequent attacks of angina):

People over 40 years old;
in individuals with the risk factors IBS;
In persons with an established diagnosis of CHD to invasive intervention (for example, septal mioectomy or alcoholic septal ablation).

Endomocardial biopsyleft or right ventricles are recommended in cases where there are doubts about the diagnosis after the clinical and instrumental examination. When identifying the characteristic pathological characteristics of the disease, a conclusion of morphological changes in myocardium is a clinical diagnosis of GKMP. With the other side, the detection of structural changes specific for any other myocardial lesion (for example, amyloidosis) makes it possible to eliminate GKMP.

In the presence of DopplerHocardiography and magnetic resonance tomography, EMB for the diagnosis of GKMP is now practically not used.

Laboratory diagnostics

In order to eliminate other most common cardiological diseases, it is necessary to carry out a biochemical blood test (lipid spectrum, myocardial necrosis biomarkers, blood serum glucose), evaluation of the functional state of the kidneys, liver and general blood tests and urine.

Differential diagnosis

Differential diagnosis is carried out with a number of diseases accompanied by the development of left ventricular hypertrophy, primarily the "heart of an athlete", acquired and congenital defects, DCMP, and with a tendency to increase blood pressure - essential arterial hypertension. Differential diagnosis with heart defects, accompanied by systolic noise, is particularly important in cases of obstructive form of GKMP. In patients with focal and ischemic changes on ECG and angiositic pain, the primary task is the differential diagnosis with IBS. When prevailing in the clinical picture of signs of stagnant heart failure in combination with a relatively small increase in the size of the heart GKMP should be differentiated from the atrial mysteries, chronic pulmonary heart and diseases flowing with restriction syndrome - constrictive pericarditis, amyloidosis, hemochromatosis and sarcoidosis of the heart and restrictive cardiotomopathy.

"Heart athlete.Differential diagnosis of the unstructive GKMM, especially with a relatively little pronounced left ventricular hypertrophy (wall thickness 13-15 mm), from the "heart of an athlete" represents complex task, often found in sports medicine. The importance of its decision is due to the fact that GKMP is the main cause of the death of young professional athletes, and therefore the formulation of such a diagnosis serves as the basis for their disqualification. The likely GKMP in these disputed cases indicates the presence of an ECG, except for signs of hypertrophy of the left ventricle, other changes. When doppler-echoche in favor of GKMM, the unusual distribution of myocardial hypertrophy, a decrease in the finite-diastolic diameter of the left ventricle is less than 45 mm, an increase in the size of the left atrium and other signs of the impaired diastolic filling of the left ventricle.

Coronary artery disease. Most often, the GKMM has to differentiate with chronic and less as sharp forms of IBS. In both cases, anginal pain in the field of heart, shortness of breath, heart rate disorders, concomitant arterial hypertension, additional tones in diastole, finely and large-scale changes and signs of ischemia to the ECG value for the diagnosis has echoca, in which part of patients has The intrinsic segmentation disorders, moderate dilatation of the left ventricle and the reduction of its emission fraction are determined. Left ventricular hypertrophy is very moderate and more often is symmetrical. The impression of the disproportionate thickening of the interventricular partition can create the presence of akinesia zones due to post-infarction cardiosclerosis in the area of \u200b\u200bthe rear wall of the left ventricle with compensatory myocardial hypertrophy. At the same time, as opposed to asymmetric hypertrophy of the interventricular partition as a form of GKMP, the hypertrophy of the partition is accompanied by hyperkinesia. In cases of noticeable dilatation of the left atrium, due to the concomitant mitral regurgitation, the Dilatation of the left ventricle is invariably notable, the dilatation of the left ventricle is unchanged. Confirm the diagnosis of GKMM allows the detection of signs of a subaartal pressure gradient. In the absence of echochegous data in favor of subaartal obstruction, differential diagnosis is significantly hampered. The only reliable method of recognition or exclusion of the IBS in such cases is X-ray-contrast coronorography. In persons of middle and older, especially in men, it is necessary to keep in mind the possibility of a combination of GKMP with IBS.

Essential arterial hypertension. For differential diagnosis, the greatest complexity is the GKMP, which flows with an increase in blood pressure, which should be distinguished from the isolated essential arterial hypertension, accompanied by the left ventricle hypertrophy with the disproportionate thickening of the interventricular partition. In favor of essential arterial hypertension, a significant and persistent increase in blood pressure is evidenced, the presence of retinopathy, as well as an increase in the thickness of intima and the media of sleepy arteries, not characteristic of patients with GKMP. Special attention should be paid to identifying signs of subaartal obstruction. In the absence of a subaartal pressure gradient on a probable GPM, in contrast to essential arterial hypertension, a significant severity of asymmetric hypertrophy of the interventricular septum is indicated with an increase in its thickness by more than 2 times compared to the rear wall of the left ventricle, as well as the detection of GKMM at least one of the 5 adult blood relatives. On the contrary, in the absence of signs of GKMP in 5 and more family members, the probability of this disease does not exceed 3%.

Complications

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

Sudden Heart Death (WSS)
- thromboembolia
- progression of chronic heart failure (CHHN).

Treatment

TO general events The limitation of significant physical exertion and prohibition of sports that can cause aggravation of myocardial hypertrophy, increasing the intraventricular gradient of pressure and risk of Sun. To prevent infectious endocarditis in situations associated with the development of bacteremia, antibiotic-porphylaxis is recommended for obstructive formas, similar to those in patients with heart defects.

The basis of drug therapy GKMM is preparations with a negative inotropic effect: β-adrenoblays and calcium channel blockers (verapamil).

β-adrenoblocators Steel first and remain to this day the most effective group of drugs used in the treatment of GKMP. They should be prescribed to patients regardless of the severity of the gradient of the intragericular pressure alone. It is preferable to refrain from the purpose of the β-aope-noretoletor blockers with internal sympathomimetic activity (PIN-dol, oxprenolol). Propranolol is prescribed at a dose of 240-320 mg per day and more (maximum daily dose - 480 mg), metoprolol - at a dose of 200 mg per day or more. Cardooselective Beta-Adrenorezp-Torov blockers with hypertrophic cardiomyopathy do not have advantages over non-selective, since in high doses the selectivity is practiced.

In the case of contraindications to the purpose of beta-adrenoreceptor blockers or the incomplete disappearance of symptoms, alternative may be calcium channel blockers. Among the calcium channel blockers with the drug selection is verapamil (isoptin, fooptin). It provides a symptomatic effect in 65-80% of patients. When appropriate, calcium channels are prescribed maximum caution in the presence of pronounced hypertrophy and very high pressure on-completely left ventricle. It should be borne in mind that the blockers of calcium channels, including verapamil, with long-term use of the Mo-Gut to increase the diastolic pressure and reduce the cardiac output. Treatment with verapamil should be started from appointment at low doses - at 20-40 mg 3 times a day, gradually increasing to a daily dose of 240-320 mg and more. Clinical improvement in the reception of verapamil is approached by increasing tolerance to physical exertion.

For the treatment of arrhythmias used antiarrhythmic drugs from which the most effective are dizeciramide and amiodar. Dizeciramide (rhythmile) relating to the antiarrhythmics of IA class has a pronounced negative inotropic effect, in patients with GKMP, it is capable of reduced the level of obstruction of the output tract of the left ventricle, has a positive effect on the diastole structure. The initial dose is usually 400 mg / day with a gradual increase of up to 800 mg. At the same time, it is necessary to control the duration of the Q-T interval on the ECG.
The only drug, against which today, the elimination of ventricular tachyrhythmia is noted, the decline in the incidence of sudden death and the improvement of the disease forecast is amiodar. Amiodarons are permanent in a daily dose of 1200 mg for 5-7 days, then in a daily dose of 800 mg and 600 mg for the 2nd and 3rd week of treatment, followed by a transition to a supporting daose dose of 200 mg.

In the treatment of GKMP with symptoms of heart failure, the following points must be taken into account:
contraindicated diuretics, Which, although the effects, tigalously reduce the stagnation in the lungs, however, can cause hypovolemia that can enhance the obstruction of the enduring path in patients! Too long-term use of diuretics can determine the reduction of shock volume and cardiac output.
Vasodilators (nitroglycerin, sodium nitroprusside) have limited use due to the possible risk of developing pronounced hypotension and reduce the size of the left ventricular polo, which may worsen the patient's condition.
Inotropic agents aimed at stimulating systolic ejection (heart glycosides and pressor amines), They may produce a non-favorable hemodynamic effect - they enhance the obstruction of the ending path and do not reduce the increased finite-diastolic pressure, can cause the development of asistolia. Nevertheless, digoxin can be used in patients with diastolic dysfunction and atrial fibrillation to reduce heart rate and / or to restore sinus rhythm.
Drug selection for CXN treatment may be Inhibi Torah ACE Because of their ability to block the renin-angiotensin system and cause the opposite development of the left ventricular hypertrophy.

In the absence of a clinical effect of active drug therapy, symptomatic patients with III-IV functional class according to the classification of the New York Association of Cardiologists with a pronounced asymmetric hypertrophy of the MZHP and a subaartal pressure gradient at rest equal to 50 mm Hg. Art. and more, shown surgical treatment (reference to treatment from i42.1)

Forecast

The unstructive forms of GKMP have, in general, a more favorable course with a smaller expression of the limitation of the functional state and a greater duration of the stabilization periods. The region is most favorable with a long asymptomatic course of the disease and an unbounded family history, especially at the highest form of GKMM. In some such cases, the disease may not be reflected on life expectancy.

Most patients with GKMM die suddenly, regardless of the possibility of the disease. An unfavorable prognosis in children, most of which are asymptomatic, is associated with a burded family history regarding sudden death. Adolescents and young and middle-aged people (from 15 to 56 years old) are the main factor that aggresses the forecast is the exposure to fainting. Patients in older people are prognostically unfavorable are shortness of breath and pain in the heart area during exercise.

5 main options for the course of the disease and outcomes are distinguished:
- stable, benign flow;
- sudden death;
- progressive flow - strengthening of shortness of shortness of breath, weakness, fatigue, pain syndrome (atypical pain, angina), the appearance of synicopal states, disorders of LV systolic dysfunction;
- "final stage" - further progression of phenomena of stagnant heart failure associated with remodeling and LV systolic dysfunction;
- Development of atrial fibrillation and complicated complications associated with it, in particular thrombometric.

Hospitalization

Indications for hospitalization

Clinically significant deterioration of the symptoms of the CHF.
- Disturbances of the rhythm of the heart: for the first time arising, hemodynamically unstable, life-degrading.
- Stop blood circulation (asistolia or ventricular fibrillation).
- Brain symptoms (syncopal, pressing states).
- Stubborn anginal pain.

Prevention

The prevention of the disease is to identify it in the early stages, which allows you to start early treatment of the disease and prevent the development of expressed myocardial hypertrophy. EchoCG should be made in obligatory in the "blood" (genetic) relatives of the patient. All other persons showed a detailed examination in the presence of similarity of manifestations: fainting, angina, etc. Screening (all in a row) ECG and ECOGG during annual dispensarization are also useful.

Information

Information

1. Treatment of heart failure. Recommendations of the Working Group on the Study of the Heart Failure of the European Society of Cardiologists. Rus. honey. journal Application. 1999.
2. Amosova E.N. Cardiomyopathy. Kiev: "Book Plus", 1999; 421 p.
3. Kushakovsky M.S. Chronic stagnant heart failure. Idiopathic myocardiopathy. St. Petersburg: "Folitan", 1998; 320 p.
4. Moiseev V.S., Sumarokov A.V., Shzyazhkin V.Yu. Cardiomyopathy. M.: Medicine 1993; 176 p.
5. Guide on Ambulatory -Poliklinic Cardiology under the Red Belenkova Yu.N, Oganova R.G., Publishing House Gootar Media, - 2007.-400 p.
6. Polyakov V.P., Nikolaevsky V.N., Picko G.A. Non-cultural and infectious diseases of the heart disease (modern aspects of clinic, diagnosis, treatment): monograph. - Samara, 2010, -355С.
7. Domestic diseases in Tinsley R. Harrison / Ed. E. Faucci, Yu. Brownwald, K. Isselbahher, J. Wilson, J. Martina, D. Kasper, S. Khauzer and D.Ligo: 7T. - M.: Practice - Mak-Grow_hill, 2005.
8. Clinical recommendations. Cardiology / Ed. Yu.N. Belenkova, R.G. Oganova. - M.: Gootar Media, 2007.
9. B.Griffin, E.Topol "Cardiology" Moscow, 2008
10. Kovalenko V.N., Nesukai E. G. Non-cultural heart disease: practical guide / ed. V. N. Kovalenko. K.: "Morion", 2001. - 480 p.

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One of the characteristic symptoms of hypertrophic cardiomyopathy is hypertrophy of the MZHP (interventricular septum). In the occurrence of this pathology there is thickening of the walls of the right or left ventricle of the heart and the interventricular partition. In itself, this condition is derived from other diseases and is characterized by the fact that the thickness of the walls of the ventricles increases.

Despite its prevalence (MZP hypertrophy is observed in more than 70% of people), it occurs most often as presptically and is detected only with very intense physical exertion. After all, the hypertrophy of the interventricular partition is her thickening and what is happening due to the reduction of the useful volume of the heart chambers. With an increase in the thickness of the heart walls of the ventricles, the size of the heart cameras is also reduced.

In practice, this all leads to a reduction in blood volume, which is ejected by the heart into the body's vascular bed. In order to provide organs with a normal amount of blood in such conditions, the heart should be reduced stronger and more often. And this, in turn, leads to its early wear and occurrence of diseases of the cardiovascular system.

Symptoms and causes of hypertrophic cardiomyopathy

A large number of people around the world lives with undiagnosed hypertrophy of MZHP, and only with increased physical exertion about its existence it becomes known. While the heart can provide a normal influx of blood to organs and systems, everything flows hidden and the person will not experience any painful symptoms or other discomfort. But on some symptoms still worth paying attention and turn to the cardiologist when they are manifest. These symptoms include:

• progressary pain;
• shortness of breath with an increase in physical activity (for example, lifting on the stairs);
• dizziness and faint;
• increased fatigue;
• tahiaritimia arising for short periods of time;
• noise in the heart with auscultation;
• labored breathing.

It is important to remember that the intravenous hypertrophy of the MZP can cause sudden death even young and physically strong people. Therefore, it is impossible to neglect the dispensary inspection of the therapist and / or cardiologist.

The reasons for the occurrence of this pathology are linked not only in the improper lifestyle. Smoking, alcohol abuse, overweight - all this becomes a factor contributing to the rise of coarse symptoms and the manifestation of negative processes in the body with an unpredictable course.

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

The consequences of the development of such deviation are dangerous.

After all, additional problems in such cases will also disrupt the conductive heart system, as well as the weakening of myocardium and the associated reduction in blood emissions during heart abbreviations.

Possible complications of hypertrophy MZP

What complications are possible in the development of the cardiopathy of the type under discussion? Everything will depend on the specific case and individual development of a person. After all, many will not recognize throughout their life about the presence of this state, and some may experience significant physical indisposition. We list the most frequently arising in the thickening of the interventricular partition consequences. So:

1. 1. Violation of cardiac rhythm in Tachycardia. Such common species like flickering arrhythmia, ventricular fibrillations and stomatricular Tachycardia Directly associated with hypertrophy of the MZP.
2. 2. Blood circulation disorders in myocardium. Symptoms arising from the violation of blood outflow from the heart muscle, there will be pain in the chest, fainting and dizziness.
3. 3. Dilatium cardiomyopathy and the associated reduction in cardiac output. The walls of cardiac chambers in the conditions of pathologically high load over time are thinned, which is the reason for the appearance of this state.
4. 4. Heart failure. Complication is very dangerous for life and in many cases ending with death.
5. 5. Sudden Heart Stop and Death.

Of course, the last two states are frightening. But, nevertheless, with a timely visit to the doctor, if any symptom of heart activities occur, a timely appeal to the doctor will help live a long and happy life.

Content

Thickening the wall of myocardium (muscular sheath of the heart) - pathological condition. AT medical practice meet different kinds Cardiomyopathy. Morphological changes in the main organ blood system They lead to a decrease in the contractile ability of the heart, there is insufficiency of blood supply.

What is hypertrophic cardiomyopathy

The disease characterized by thickening (hypertrophy) of the left (rare right) ventricular heart is called hypertrophic cardiomyopathy (GKMP). Muscular myocardial fibers are chaotic - it is characteristic feature Diseases. In most cases, the asymmetricness of the thickening is observed, hypertrophy of the interventricular partition develops.

Pathology is characterized by a decrease in the volume of the ventricle, a violation of the pump function. The heart has to shrink often to deliver a sufficient amount of blood to organs. The consequence of these changes is the violation of the heart rhythm, the emergence of heart failure. 30-50 years - average age of patients with diagnosed hypertrophic cardiomyopathy. The disease is more common in men. The pathological state is recorded in 0.2-1.1% of the population.

The reasons

GKMP is a hereditary disease. Arises as a result of the generation of genes. The type of transmission of modified hereditary structures is an autosomal dominant. Pathology is not only congenital. In some cases, mutations arise under the action of adverse environmental factors. The effects of changing genetic code are as follows:

• the biological synthesis of contracting proteins of myocardium is disturbed;
• muscle fibers have an improper location, structure;
• muscular tissue is partially replaced by the connective, fibrosis of myocardium is developing;
• changed cardiomyocytes (muscular shell cells) work is not coordinated, with an increased load;
• muscular fibers thicken, myocardial hypertrophy occurs.

To the thickening of the muscular shell (compensatory hypertrophy), one of two pathological processes leads:

1. Disruption of myocardial diastolic function. In the period of relaxation of the heart (diastole) of the ventricle filled with blood not enough due to poor myocardial tensions. This leads to an increase in diastolic pressure.
2. Obstruction (violation of passability) of the output path of the left ventricle. There is hypertrophy of the myocardium interventricular partition. Bleeding is hampered due to violation of mobility of the mitral valve flap. At the time of blood release between the left ventricular cavity and the initial degree of the aorta, the systolic pressure drop occurs. For this reason, some of the blood is delayed in the heart. As a result, the final diastolic pressure of the left ventricle rises. Hypertrophy of myocardium, dilatation (expansion) of the left atrium - the effect of compensatory hyperfunction.

Classification

Criteria based on the classification of the disease are different. Allocate the following types of illness:

Criterion		Common diagnoses: asymmetric hypertrophy of the interventricular partition; hypertrophic obstructive cardiomyopathy; asymmetric Heart Top Hypertrophy (Apical)
Localization	Hypertrophy of the right ventricle or left ventricle
Features of the formation of thickening	Asymmetric, concentric (or symmetric)
Changed structures	Hypertrophy of the interventricular partition, the tops of the heart, the front-winding wall, the rear wall
The presence of a gradient (difference) of systolic pressure in the left ventricle	Obstructive, unstructive
The degree of thickening myocardium	Moderate - 15-20 mm, average - 21-25 mm, pronounced - more than 25 mm

Taking into account the prevailing complaints of patients allocate nine forms of pathology. In the presence of general symptoms, each GKMM variant has specific features. Clinical forms are as follows:

• lightning room;
• pseudochapala;
• arrhythmic;
• cardialgic;
• low-power;
• vegetodistonic;
• infarct-like;
• decompensation;
• mixed.

Clinical and physiological classification allocates four stages of development of the disease. The main criterion is the difference in systolic pressure in the output path of the left ventricle (VTL) and in the aorta:

• The first stage is a pressure indicator in VTLZH not more than 25 mm Hg. Art. There are no complaints of deterioration in the patient.
• The second stage is a pressure gradient of about 36 mm Hg. Art. The condition worsens during exercise.
• Third stage - the pressure difference is up to 44 mm Hg. Art. There is a shortness of breath, angina develops.
• The fourth stage is a systolic pressure gradient in VTLZH more than 88 mm Hg. Art. There is a breakdown of blood circulation, a sudden death is possible.

Symptoms of hypertrophic cardiomyopathy

Disease for a long time may not manifest. Hypertrophic cardiomyopathy is the main cause of the death of young athletes who did not guesses hereditary disease. 30% of patients with GTCP do not have complaints and have no deterioration in the overall state. Symptoms of pathology are as follows:

• fainting, dizziness, shortness of breath, cardialia, angina and other states associated with small blood release syndrome;
• heart failure left ventricle;
• violations of the rhythm of heart abbreviations (extrasystoles, paroxysms, arrhythmias);
• sudden death (against the background of the complete absence of symptoms);
• the emergence of complications - infectious endocarditis, thromboembolism.

Diagnostics

The first signs of the disease are manifested in childhoodBut in most cases it is diagnosed in adolescent or in patients 30-40 years. Based on the physical examination (evaluation of the external state), the doctor puts the primary diagnosis. Inspection helps to identify the expansion of the heart boundaries, listen to the characteristic systolic noise if there is an obstructive form of the disease, the second tone accent is possible on the pulmonary artery. When examining the cervical veins into hypertrophy, the poor reduction of the right ventricle indicates a well-pronted wave A.

Additional diagnostic methods include:

• Electrocardiography. In the presence of pathology, the ECG is never normal. The study allows you to establish an increase in cardiac cameras, violation of conductivity and abbreviation frequency.
• Chest x-ray. Helps to identify changes in the sizes of atria and ventricles.
• Echocardiography. The main method for identifying the localization of the thickening of the heart wall, the degree of obstruction of blood flow, diastolic dysfunction.
• Monitoring electrocardiogram during the day, using physical exertion. The method is important for the prevention of sudden death, forecast of the disease, identifying heart rate disorders.
• Radiological methods. Ventriculography is carried out (the study of the heart with the introduction of a contrast substance), magnetic resonance tomography (MRI). Apply them in difficult cases for identifying and accurate assessment of pathological changes.
• Genetic diagnostics. The most important method for assessing the forecast of the disease. The analysis of the genotype is carried out in the patient and members of his family.

Treatment of hypertrophic cardiomyopathy

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

• reducing the severity of symptoms and clinical manifestations of pathology;
• improving the "quality of life" of the patient, improving the functional ability;
• ensuring a positive forecast of the disease;
• prevention of cases of sudden death and progression of the disease.

Medical therapy

Patients with symptoms GKMP recommended restriction of physical exertion. This rule should be strictly carried out by patients with obstructive form of the disease. Loads provoke the development of arrhythmias, fainting, increasing the pressure gradient VTLZH. To facilitate the state with moderately pronounced symptoms, GKMP prescribed drugs of different pharmacological groups:

• Beta blockers (propranolol, metoprolol, atenolol) or calcium channels (verapamil) blockers. They reduce the heart rate, lengthen the diastole (phase of relaxation), improve the filling of ventricles with blood, reduce diastolic pressure.
• Calcium antagonists (fanoptein, amiodar, cardil). Drugs reduce the amount of calcium in coronary arteries, improve the relaxation of structures (diastole), stimulate the reduction of myocardium.
• Anticoagulants (Phoenindion, Heparin, Bivalirudin). Drugs reduce the risk of thromboembolism.
• Diuretics (Furosemid, Indapamed), ACE inhibitors (captopril, Ramipril, Fozinopril). Preparations are recommended for patients with heart failure.
• Antiarrhythmic drugs (Dizeciramide, amiodaron).

Under GKMM, the reception of cardiac glycosides, nifedipine, nitrites is contraindicated. These drugs contribute to the development of obstruction.

Operational intervention

Cardiac surgical treatment is advisable in the absence of effectiveness of pharmacological preparations. Operational intervention is shown in patients who have a pressure difference between the left ventricle (LV) and the aorta of more than 50 mm Hg. Art. At rest and during exercise. In order to facilitate the patient's condition, the following surgical techniques are used:

• Crystal septal meshtomy (CME). It is recommended for patients who, during exercise, fainting, shortness of breath, chest pain. The essence of the operation is to remove part of the interventricular partition. This manipulation provides a good reduction in LV and free pushing blood in the aorta.
• Percutaneous alcohol ablation. The operation is prescribed to patients with contraindications to the CME, the elderly patients in which inadequate indicators of blood pressure are observed under load conditions. The hypertrophized interventricular partition is introduced sclerosing substances (for example, alcohol solutions).
• Two-chamber electrocardialism. The technique is used for patients with contraindications to carry out surgical intervention. Manipulation improves the work of the heart, contributes to an increase in cardiac output.
• Implantation of artificial mitral valve. Prosthetics is recommended to patients whose poor blood outflow occurs not due to the thickening of the interventricular septum, but as a result of the valve turning into the lumen of the aorta.
• Installation of ICD (implantable cardioverter-defibrillator). Indications for such a procedure are the high risk of sudden death, transferred to the stop of the heart, resistant ventricular tachycardia. For the installation of the ICD in the plug-in area, the electrodes are entered through vein (thereof 1-3 may be, depending on the model of the pacemaker) and are installed under the radiographic control inside the heart.
• Heart transplantation. It is prescribed to patients at the last stage of heart failure, in the absence of the effect of drug treatment.

Surgical operations significantly improve the patient's condition, increase portability of physical exertion. Operational treatment does not protect against the further development of the pathological thickening of myocardium and complications. In the postoperative period, the patient needs to regularly undergo surveys using hardware diagnostic techniques and to make pharmacological preparations appointed by a doctor.

Forecast

Hypertrophic cardiomyopathy has different development options. The unstructive form proceeds steadily, without pronounced symptoms. The consequence of the prolonged development of the disease is heart failure. In 5-10% of cases, the thickening of the myocardial wall ceases independently, the same percentage of the likelihood of transformation of hypertrophic cardiomyopathy into dilatation (stretching of the heart cavities).

Mortality in the absence of treatment is observed in 3-8% of cases. Half patients comes a sudden death caused by heavy ventricular arrhythmias, a complete blockade of the heart, an acute myocardial infarction. In 15-25% - the coronary atherosclerosis develops. Infectious endocarditis, in which mitral, aortic valves are affected, arises as a complication of pathology in 9% of patients.

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