Myocardial disease, which is often genetically determined (mutation of the gene encoding one of the cardiac sarcomere proteins), and is characterized by myocardial hypertrophy, mainly of the left ventricle, often with asymmetric ventricular septal hypertrophy and, as a rule, with preserved systolic function. According to the current classification, HCMP also includes genetically determined syndromes and systemic diseases in which myocardial hypertrophy develops, incl. amyloidosis and glycogenosis.

CLINICAL PICTURE AND TYPICAL COURSE

1. Subjective symptoms: shortness of breath during physical activity(common symptom), orthopnea, angina pectoris, syncope or pre-syncope conditions (especially in the form with narrowing of the left ventricular outflow tract).

2. Objective symptoms: systolic "cat's purr" over the apex of the heart, diffuse apex beat, sometimes - its bifurcation, III (only with left ventricular failure) or IV tone (mainly in young people), early systolic click (indicates a significant narrowing outflow tract of the left ventricle), systolic murmur, usually crescendo-decrescendo, sometimes - fast biphasic peripheral pulse.

3. Typical course: depends on the degree of myocardial hypertrophy, the magnitude of the gradient in the output tract, the tendency to arrhythmias (especially atrial fibrillation and ventricular arrhythmias). Patients often live to an advanced age, but there are also cases sudden death at a young age (as the first manifestation of HCM) and heart failure. Risk factors for sudden death:

• 1) the appearance of a sudden circulatory arrest or persistent ventricular tachycardia;
• 2) sudden cardiac death in a relative of the 1st degree;
• 3) recent unexplained syncope;
• 4) left ventricular wall thickness ≥ 30 mm;

5) pathological reaction blood pressure(increase<20 мм рт. Ст. Или снижение ≥ 20 мм рт. Ст.) Во время физической нагрузки, или эпизоды неустойчивой желудочковой тахикардии при холтеровском исследовании или электрокардиографическом тесте с физической нагрузкой, и наличие других дополнительных факторов. которые влияют на риск (значительного сужения выходного тракта левого желудочка, эффекта позднего усиления контрастирования при МРТ,>1 gene mutation).

Diagnostics

ancillary studies

1. ECG: pathological Q wave, especially in leads from the lower and lateral wall, livogram, irregularly shaped P wave (indicates an increase in the left atrium or both atria), a deep negative T wave in leads V2-V4 (with the upper form of HCM) .

2. WG chest: may detect enlargement of the left ventricle or both ventricles and the left atrium, especially with concomitant mitral valve insufficiency.

3. Echocardiography: significant myocardial hypertrophy, in most cases generalized, usually with damage to the interventricular septum, as well as the anterior and lateral walls. In some patients, hypertrophy of only the basal parts of the interventricular septum is observed, which leads to narrowing of the left ventricular outflow tract, which in 25% of cases is accompanied by anterior systole movement of the mitral valve cusps and its insufficiency. In 1/4 of cases, there is a gradient between the original left ventricular tract and the aorta (gradient > 30 mm Hg. Has prognostic value). The examination is recommended for preliminary evaluation of a patient with suspected HCM and as a screening study in relatives of patients with HCM.

4. Electrocardiographic exercise test: to assess the risk of sudden cardiac death in patients with HCM.

7. 24-hour Holter ECG monitoring: to detect possible ventricular tachycardias and evaluate indications for ICD implantation.

8. Coronary angiography: in case of chest pain, in order to exclude concomitant coronary disease.

diagnostic criteria

Based on the detection of myocardial hypertrophy during echocardiography and the exclusion of its other causes, primarily arterial hypertension and stenosis aortic valve.

differential diagnosis

Arterial hypertension (in the case of HCM with symmetrical left ventricular hypertrophy), aortic valve insufficiency (especially stenosis), myocardial infarction, Fabry disease, recent myocardial infarction (with increased concentration of cardiac troponins).

Pharmacological treatment

1. Patients without subjective symptoms: observation.

2. Patients with subjective symptoms: β-blockers (eg, bisoprolol 5–10 mg/day, metoprolol 100–200 mg/day, propranolol up to 480 mg/day, especially in patients with a gradient in the left ventricular outflow tract after exercise) ; increase the dose gradually, depending on the effectiveness and tolerability of drugs (continuous monitoring of blood pressure, pulse and ECG is necessary); in case of failure → verapamil 120-480 mg/day or diltiazem 180-360 mg/day, caution in patients with narrowing of the outflow tract (in these patients do not use nifedipine, nitroglycerin, digitalis glycosides and ACE inhibitors).

3. Patients with heart failure pharmacological treatment as in DCM.

4. Atrial fibrillation: try to restore and maintain sinus rhythm with amiodarone (also indicated for ventricular arrhythmias) or sotalol. In patients with permanent atrial fibrillation, anticoagulant therapy is indicated.

invasive treatment

1. Surgical resection narrowing the left ventricular outflow tract of part of the interventricular septum (myectomy, Morrow operation): in patients with an instantaneous gradient in the outflow tract > 50 mmHg. Art. (at rest or during exercise) and severe symptoms that limit vital activity, usually dyspnea on exertion and chest pain, not responding to pharmacological treatment.

2. Percutaneous alcohol ablation of the septum: the introduction of pure alcohol into the perforating septal branch in order to develop myocardial infarction in the proximal part of the interventricular septum; indications are the same as for myectomy; the effectiveness is similar to that of surgical treatment. Not recommended for patients aged<40 г. которым можно выполнить миектомию.

3. Dual chamber pacing: to enable intensification of pharmacological treatment; consider indications in patients in whom myectomy or alcohol ablation cannot be performed; not recommended for severe HCM with narrowed left ventricular outflow tract.

4. Heart transplantation. with terminal form of heart failure, does not respond to treatment.

5. Implantation of a cardioverter-defibrillator: in patients with a significant risk of sudden death (primary prevention), as well as in patients after a cardiac arrest or with persistent, spontaneously occurring ventricular tachycardia (secondary prevention).

In patients at high risk of death, perform an annual exercise ECG and Holter ECG monitoring; in patients weighed down by an insignificant risk - every 3-5 years. If a pathogenic mutation is detected in an asymptomatic person, periodic follow-up examinations (including also ECG and transthoracic echocardiography) every 5 years in adults, and every 12-18 months. in children.

The most interesting news

Hypertrophic cardiomyopathy. Diagnosis and treatment of hypertrophic cardiomyopathy.

It is characterized by hypertrophy of the left and / or right ventricle with diffuse or segmental thickening of their walls and the interventricular septum. The cavity of the left ventricle is normal or reduced. This condition may be accompanied by impaired systolic or diastolic ventricular function.

Asymmetrical hypertrophy of the interventricular septum occurs in 70% of patients, concentric left ventricular hypertrophy - in 30%. In the latter form, the right ventricle is often also involved in the process (-75%); almost a third of children have Noonan syndrome.

Hypertrophic cardiomyopathy can be presented as a non-obstructive or obstructive (40%) form, which is determined by the presence of a gradient in the output section of the left ventricle of more than 15 mm Hg. Art. The obstructive form of HCM has a worse prognosis, as it leads to an increased load on the ventricular wall, myocardial ischemia, cell death and their replacement with fibrous tissue.

Intraventricular narrowing can be located directly in the subaortic or in the middle section of the left ventricle. As a rule, obstruction is not fixed, but dynamic. At an older age, it is detected using provocative tests (with physical activity, with inotropic drugs). In the subaortic region, the narrowing is formed due to the anterior leaflet of the mitral valve, which, under the influence of a powerful blood flow into systole, makes an anterior movement and comes into contact with the hypertrophied interventricular septum. This phenomenon, in addition to exit obstruction, leads to incomplete closure of the mitral valve leaflets and to regurgitation mainly in the posterior part of the left atrium. When the jet of regurgitation is directed anteriorly or centrally, one can assume an independent lesion of the mitral valve (myxomatous degeneration, fibrosis, etc.). In about 5% of cases, the narrowing located in the middle part of the ventricle is associated with hypertrophy and abnormal localization of the papillary muscles. In these cases, mitral regurgitation is not detected.

Considering the fact what is hypertrophic cardiomyopathy in a significant part of cases, it has a family character and is transmitted by an autosomal dominant type; if a patient with this pathology is identified, an examination of the next of kin is necessary.

Clinical symptoms of hypertrophic cardiomyopathy.

Foundations for fetal examination similar to those in dilated cardiomyopathy. HCM during fetal echocardiography is diagnosed if the thickness of the ventricular wall exceeds two standard deviations for a given gestational age (see Appendix 1). In 30-50% of fetuses, systolic and diastolic dysfunction and regurgitation of the atrioventricular valves are found.

After birth symptoms may be scarce. Auscultation in half of the children is determined by systolic murmur of varying intensity. Rhythm disturbances are possible, mainly supraventricular tachycardia. If the dysfunction of the heart was manifested in utero, the main symptoms in newborns will be shortness of breath, tachycardia, fluid retention. With biventricular obstruction, the occurrence of cyanosis and the rapid progression of the disease are not excluded.

In general, the manifestation pathology in the neonatal period indicates its severity and is accompanied by a poor prognosis. The overall mortality, including the prenatal period, is more than 50%.

Electrocardiography. As a rule, during the neonatal period, ECG changes are minimal. There may be signs of hypertrophy of the left and right ventricles, nonspecific disorders of repolarization processes.

Chest x-ray. The shadow of the heart and pulmonary pattern change with the development of congestive heart failure. In its absence, pathological changes may not be.

echocardiography. The study most often reveals asymmetric hypertrophy of the interventricular septum, and in about a third of patients - concentric hypertrophy of the left ventricle. Additionally, you can set the anterior systolic movement of the mitral leaflet, the presence of midventricular obstruction, mitral regurgitation.

In most cases (about 80%), diastolic ventricular dysfunction is determined - a decrease in the VE peak and an increase in the VA peak.

Treatment of hypertrophic cardiomyopathy.

The main drugs used for treatment of hypertrophic cardiomyopathy in adults, are β-blockers, disopyramide, calcium channel blockers with a negative inotropic effect. The result of their use is also a decrease in heart rate, lengthening of diastole and improvement of conditions for passive filling of the ventricles. However, the effect of these drugs in newborns has not been studied enough. For example, verapamil is known to increase pulmonary artery pressure, and its intravenous administration in infants can lead to sudden death. β-blockers of the fourth generation seem to be more promising. Cases of their use in infants are few, but at an older age they are quite effective. Additionally, you can use drugs that improve the energy metabolism of cells.

Should be cautioned against using vasodilators. ACE inhibitors or digoxin in the treatment of HCM, as these drugs can provoke ventricular obstruction.

Surgical intervention with hypertrophic cardiomyopathy consists in resection or dissection of a hypertrophied interventricular septum and is performed at an older age.

Hypertrophic cardiomyopathy - symptoms, diagnosis and treatment

Hypertrophic cardiomyopathy is a congenital or acquired disease characterized by marked hypertrophy of the ventricular myocardium with diastolic dysfunction and cardiac arrhythmias.

Causes of hypertrophic cardiomyopathy

The disease can develop as a result of a predisposition to it, high blood pressure and physiological aging of the body.

Symptoms of hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy is asymptomatic or minimal in many people, but some may show signs of the disease and progress.

Symptoms of hypertrophic cardiomyopathy include:

high pressure and pain in the chest that occurs after eating or exercise;

• dyspnea;
• fast fatiguability;
• cardiopalmus;
• fainting.

Death occurs in a minority of patients.

Diagnosis of hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy is diagnosed on the basis of the medical history (complaints of the patient, taking into account the influence of heredity), the results of the ecocardiogram and physiological studies. It is also possible to conduct additional studies, which include a chest x-ray, ECG, blood test, magnetic resonance, computed tomography, cardiac catheterization, exercise test.

Treatment of hypertrophic cardiomyopathy

In the treatment of this disease, the main symptoms are corrected, and life-threatening complications are prevented. Patients are prescribed medications that relax the heart and improve circulation. Traditional medicines are considered to be two classes of drugs - calcium channel blockers and beta-blockers. For the treatment of arrhythmia, drugs are used that reduce its manifestations and control the heart rate.

Signs of hypertrophic cardiomyopathy without symptoms of vascular blockage are treated with drugs. The treatment of heart failure is to control it with the help of special medications.

In case of failure of conservative treatment, surgical intervention is indicated. Surgical procedures for hypertrophic cardiomyopathy include ethanol ablation, septal myectomy, or implantation of an electrical defibrillator.

In hypertrophic cardiomyopathy, lifestyle changes are also recommended.

You must follow the diet prescribed by your doctor. If there are no restrictions on drinking, you should drink six to eight glasses of water per day, increase fluid intake during the heat. Patients with heart failure should limit their salt and fluid intake. You should consult your doctor about the use of caffeinated foods and alcoholic beverages.

As for physical activity, this issue should be discussed with your doctor. Generally, light aerobic exercise is not contraindicated for people with cardiomyopathy, but heavy lifting is highly discouraged.

Patients suffering from hypertrophic cardiomyopathy should visit a cardiologist annually. If the disease is newly diagnosed, then the frequency of visits may be increased.

Risk of sudden death

Among patients with hypertrophic cardiomyopathy, only a small proportion have an increased risk of sudden death. This group includes people with poor heredity; arrhythmia with a high heart rate; strongly rising pressure during physical exertion; with repeated cases of fainting; poor heart function and severe disease symptoms.

If you have two or more symptoms, you should immediately consult a doctor.

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

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

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

Symptoms and Causes of Hypertrophic Cardiomyopathy

A large number of people around the world live with undiagnosed IVS hypertrophy, and only with increased physical exertion does its existence become known. As long as the heart can provide normal blood flow to organs and systems, everything is hidden and the person will not experience any painful symptoms or other discomfort. But it is still worth paying attention to some symptoms and contacting a cardiologist when they appear. These symptoms include:

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

It is important to remember that undetected IVS hypertrophy can cause sudden death even in young and physically strong people. Therefore, you can not neglect the dispensary examination by a therapist and / or a cardiologist.

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

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

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

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

Possible complications of IVS hypertrophy

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

1. 1. Violation of the heart rhythm by the type of tachycardia. Common types such as atrial fibrillation, ventricular fibrillation, and ventricular tachycardia are directly related to IVS hypertrophy.
2. 2. Violations of blood circulation in the myocardium. Symptoms that occur when there is a violation of the outflow of blood from the heart muscle will be chest pain, fainting and dizziness.
3. 3. Dilated cardiomyopathy and the associated decrease in cardiac output. The walls of the heart chambers under conditions of pathologically high load become thinner over time, which is the cause of this condition.
4. 4. Heart failure. The complication is very life-threatening and in many cases ends in death.
5. 5. Sudden cardiac arrest and death.

Of course, the last two states are awesome. But, nevertheless, with a timely visit to the doctor, if any symptom of a violation of cardiac activity occurs, a timely visit to the doctor will help to live a long and happy life.

It's important to know! An effective tool for normalization work of the heart and cleaning of blood vessels exists! …

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

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

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

Symptoms and Causes of Hypertrophic Cardiomyopathy

A large number of people around the world live with undiagnosed IVS hypertrophy, and only with increased physical exertion does its existence become known. As long as the heart can provide normal blood flow to organs and systems, everything is hidden and the person will not experience any painful symptoms or other discomfort. But it is still worth paying attention to some symptoms and contacting a cardiologist when they appear. These symptoms include:

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

It is important to remember that undetected IVS hypertrophy can cause sudden death even in young and physically strong people. Therefore, you can not neglect the dispensary examination by a therapist and / or a cardiologist.

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

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

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

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

Possible complications of IVS hypertrophy

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

1. Violation of the heart rhythm by the type of tachycardia. Common types such as atrial fibrillation, ventricular fibrillation, and ventricular tachycardia are directly related to IVS hypertrophy. 2. Violations of blood circulation in the myocardium. Symptoms that occur when there is a violation of the outflow of blood from the heart muscle will be chest pain, fainting and dizziness. 3. Dilated cardiomyopathy and the associated decrease in cardiac output. The walls of the heart chambers under conditions of pathologically high load become thinner over time, which is the cause of this condition. 4. Heart failure. The complication is very life-threatening and in many cases ends in death. 5. Sudden cardiac arrest and death.

Of course, the last two states are awesome. But, nevertheless, with a timely visit to the doctor, if any symptom of a violation of cardiac activity occurs, a timely visit to the doctor will help to live a long and happy life.

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Myocardial hypertrophy (hypertrophic cardiomyopathy) is a significant thickening and enlargement of the walls of the left ventricle of the heart. Its cavity inside is not expanded. In most cases, thickening of the interventricular septa is also possible.

Due to thickening, the heart muscle becomes less extensible. The myocardium can be thickened over the entire surface or in some areas, it all depends on the course of the disease:

• If the myocardium hypertrophies mainly under the aortic origin, narrowing of the left ventricular outlet may occur. In this case, the thickening of the inner shell of the heart occurs, the valves are disrupted. In most cases, this occurs with uneven thickening.
• Asymmetric thickening of the septum is possible without violations of the valvular apparatus and a decrease in the output from the left ventricle.
• The occurrence of apical hypertrophic cardiomyopathy occurs as a result of an increase in the muscle at the apex of the heart.
• Myocardial hypertrophy with symmetrical circular hypertrophy of the left ventricle.

Disease history

Hypertrophic cardiomyopathy has been known since the mid-19th century. It was only in 1958 that the English scientist R. Teare could describe it in detail.

Significant progress in the study of the disease was the introduction of some non-invasive methods of research, when we learned about the existence of obstructions in the outflow tract and impaired distolic function.

This was reflected in the corresponding names of the disease: "idiopathic hypertrophic subaortic stenosis", "subaortic muscular stenosis", "hypertrophic obstructive cardiomyopathy". Today, the term "hypertrophic cardiomyopathy" is universal and generally accepted.

With the widespread introduction of ECHO KG studies, it was found that the number of patients with myocardial hypertrophy is much greater than was thought in the 70s. Every year, 3-8% of patients with this disease die. And every year the death rate is increasing.

Prevalence and significance

Most often, people aged 20-40 suffer from myocardial hypertrophy, men are about twice as likely. Flowing very diversely, progressing, the disease does not always manifest itself immediately. In rare cases, from the very beginning of the course of the disease, the patient's condition is severe and the risk of sudden death is quite high.

The frequency of hypertrophic cardiomyopathy is about 0.2%. Mortality ranges from 2 to 8%. The main cause of death is sudden cardiac death and life-threatening cardiac arrhythmias. The main reason is hereditary predisposition. If the relatives did not suffer from this disease, it is believed that there was a mutation in the genes of the proteins of the heart muscle.

It is possible to diagnose the disease at any age: from birth to old age, but most often patients are young people of working age. The prevalence of myocardial hypertrophy does not depend on gender and race.

In 5-10% of all registered patients with a long course of the disease, a transition to heart failure is possible. In some cases, in the same number of patients, an independent regression of hypertrophy is possible, a transition from a hypertrophic to a dilated form. The same number of cases account for emerging complications in the form of infectious endocarditis.

Without appropriate treatment, mortality is up to 8%. In half of the cases, death occurs as a result of acute myocardial infarction, ventricular fibrillation and complete atrioventricular heart block.

Classification

In accordance with the localization of hypertrophy, myocardial hypertrophy is distinguished:

• left ventricle (asymmetric and symmetrical hypertrophy);
• right stomach.

Basically, asymmetric hypertrophy of the interventricular septum is detected on the entire surface or in some of its departments. Less often, hypertrophy of the apex of the heart, anterolateral or posterior wall can be found. In 30% of cases, there is a proportion of symmetrical hypertrophy.

Given the gradient of systolic pressure in the left ventricle, hypertrophic cardiomyopathy is distinguished:

• obstructive;
• non-obstructive.

The non-obstructive form of myocardial hypertrophy includes, as a rule, symmetrical hypertrophy of the left ventricle.

Asymmetric hypertrophy can refer to both obstructive and non-obstructive forms. Apical hypertrophy mainly refers to the non-obstructive variant.

Depending on the degree of thickening of the heart muscle, hypertrophy is distinguished:

• moderate (up to 20 mm);
• medium (21-25 mm);
• pronounced (more than 25 mm).

Based on clinical and physiological classifications, 4 stages of myocardial hypertrophy are distinguished:

• I - pressure gradient at the outlet of the left ventricle, not more than 25 mm Hg. Art. (no complaints);
• II - the gradient increases to 36 mm Hg. Art. (the appearance of complaints during physical exertion);
• III - the gradient increases to 44 mm Hg. Art. (appear shortness of breath and angina pectoris);
• IV - gradient above 80 mm Hg. Art. (impaired hemodynamics, sudden death is possible).

Left atrial hypertrophy is a disease in which thickening of the left ventricle of the heart occurs, due to which the surface loses its elasticity.

If the sealing of the cardiac septum has occurred unevenly, there may also be disturbances in the work of the aortic and mitral valves of the heart.

Today, the criterion for hypertrophy is myocardial thickening of 1.5 cm or more. This disease is by far the leading cause of early death in young athletes.

Left ventricular hypertrophy or cardiomyopathy is a very common heart lesion in patients diagnosed with hypertension. This is a rather dangerous disease, since often its final stage in 4% of all cases is fatal.

What it is?

Hypertrophy implies thickening of the walls of the left ventricle and this is not due to the peculiarities of the internal space. The septum between the ventricles changes, tissue elasticity is lost.

Hypertrophic cardiomyopathy (HCM) is an isolated lesion (deformation) of the heart muscle, in particular, thickening of the wall of the ventricle (usually the left one). At the same time, the volume of its cavity remains normal (less often, it decreases), but the diastolic function, that is, the flow of blood to the heart, worsens significantly. Why does arrhythmia occur? The disease is primary.

Hypertrophic cardiomyopathy causes damage to the heart

HCM is an inherited cardiovascular disease. It occurs in 1 case out of 500, more often among the working population. However, spontaneous mutations in old age (non-classical Fabry disease) and early onset in children are possible.

Causes of pathology

The development of the disease is associated with an abnormal arrangement of muscle fibers, which is caused by gene mutations (changes in the cells responsible for contractile proteins). This is an autosomal dominant pathology, often asymmetric manifestations. In 60% of cases, hypertrophy of the interventricular septum (IVS) is noted, less often - the apex of the organ, sometimes - the middle segments.

To date, clinical medicine has identified more than 400 possible mutations - causes among genes:

• β-myosin, protein C, β light and heavy chains;

Hypertrophy of the interventricular septum is a fairly common pathology.

• troponin T, C, I;
• potassium channels;
• α-actin;
• protein kinase type A;
• titin.

In the first two groups, deformations occur most often. It is the troponin T gene that is responsible for the onset of the manifestations of the disease (the age of the person), the clinical signs of the course and the life span. Pathology has no age and gender preferences, for a long time it may not make itself felt.

The genes of healthy parents can mutate during pregnancy or in the later life of the child under the influence of negative factors: radiation, ionization, infections, bad habits (smoking, alcohol, use of other surfactants).

Smoking and alcoholism of pregnant women can provoke a gene mutation in a child

Disease classification

Hypertrophic cardiomyopathies are divided according to several criteria. All of them are shown in the table.

Hypertrophic obstructive cardiomyopathy affects the aorta, cavity and organ walls, mitral valve. Violations of the work of these elements are reflected by a number of signs.

Cardiomyopathy can affect different zones and areas of the heart

Characteristic symptoms

To general clinical signs include:

• Manifestations of heart failure (90% of cases): shortness of breath, fatigue, nocturnal attacks of asphyxia. chest pain (pressive, squeezing) that does not go away after taking painkillers. Caused by an increase in systolic blood pressure.
• Obvious, tangible disruptions in the work of the heart (frequent and excessive, or vice versa, rare contraction, interruptions. 70% of cases).
• Dizziness, fainting and pre-syncope (syndrome of small ejection. Frequency 20-25%).
• Ischemia caused by oxygen starvation due to vascular deformation, impaired blood circulation, constant tension in the muscles, walls of capillaries and ventricles.
• In 30% of cases, sudden death (loss of consciousness for more than an hour) is the only sign of an ongoing illness.

With this pathology, dizziness and fainting are often observed.

Symptoms can increase with physical exertion, stress (the moment of defecation), a sharp change in position to a vertical one (slopes). Hypertrophic cardiomyopathy has a number of risks and complications.

Possible Complications

The worst possible prognosis is sudden cardiac death (1-6% per year, 60% of which are at rest). In 5-10%, development is noted systolic dysfunction ventricle. Complications also include infective endocarditis and thromboembolism, heart attack, and stroke.

Hypertrophy of the IVS and obstruction of the outflow tract of the ventricle (35-50% of cases) are dangerous with the occurrence of life-threatening arrhythmias and arterial hypotension. Due to the enlarged septum, the valve rises to the surface and, at the moment of contraction of the ventricles, is attracted by the blood flow, which interferes with the normal circulation of the latter.

This disease can cause manifestations of hypotension, when the pressure drops to a critically low level.

Most susceptible to SCD (sudden cardiac death) people hard work and professional athletes. The risk group includes people suffering from obesity and other diseases, such as ischemia.

Diagnosis of the disease

For HCM during diagnostic procedures, thickening of the wall of one or both ventricles from 1.5 cm or more, diastolic disturbance is characteristic. In addition, there is a replacement of muscle tissue with fibrous tissue, anomalies in the vessels and the entire myocardium. Diagnosis and treatment of hypertrophic cardiomyopathy is carried out in several stages.

Initial examination (available at home):

• The study of the anamnesis: the nature of the course of the disease, the first manifestation, what is the reason for the increase in symptoms, whether someone similar suffers in the family, whether deaths have been recorded.
• Visual examination of the skin for the presence of painful whiteness or cyanosis.

The doctor at the reception must listen to the patient for the presence of systolic heart murmurs

• Percussion of the heart (determination of changes in size), auscultation (determination of systolic murmurs), pressure measurement.

Secondary examination (in a hospital setting):

• General and chemical analysis of blood, urine, feces, coagulogram (study of coagulability and blood clots). Allows you to determine the presence of adjacent inflammation and general state organism.
• Echocardiography (EchoCG). Detects murmurs, with HCM there are systolic murmurs over the aorta. Determines heart defects, cavity sizes and wall thickness (there is a decrease in the cavity of the ventricle in combination with compaction of the IVS and the wall). If it is impossible to use the method (for example, the patient is obese), radionuclide ventriculography (contrast injection into the blood) is performed.
• Electrocardiography (ECG). Shows heart rate, electrical conduction and ventricular size.

An ECG is required to make an accurate diagnosis.

• Daily ECG monitoring. It helps to track the dynamics of the disease and the effectiveness of treatment.
• Load tests are specially organized laboratory experiments on simulators to identify the features of hypertrophy and endurance of the body. Help make recommendations for rehabilitation.
• MRI (magnetic resonance imaging). Shows in detail the condition of all internal organs.
• X-ray. Determines the size of the heart (with HCM - normal or slightly enlarged) and the presence of blood stasis. Additionally excludes other inflammations and diseases.
• Gene testing. Detects a mutated gene, is carried out with the participation of the patient's relatives.

If after this complex the diagnosis is still inaccurate, then a biopsy (muscle tissue sampling) and catheterization (measurement of pressure from the inside through the tubes) are performed.

In some cases, a heart biopsy is needed.

Considering the fact that there may be no other manifestations before sudden death, it is recommended to undergo scheduled examinations by a cardiologist or a cardiac surgeon, a general practitioner. In 0.5% of cases, the pathology under consideration is detected in the process.

Treatment Methods

Treatment of hypertrophic cardiomyopathy is conservative (medication) and surgical. With a safe and stable course of the disease without signs of heart failure: regular monitoring, exclusion of negative factors (malnutrition, bad habits, hypothermia, physical and emotional overwork). These same recommendations are common to all types.

If there is a risk of SCD (various arrhythmias) - surgical implantation (an electrical device that monitors the heartbeat, which, when weakened, sends an impulse). TO surgical treatment includes myectomy (removal of part of the septum from the inside), ethanol ablation (injection with a syringe without incision of the chest alcohol solution into the septum, cells die, IVS decreases, blood flow is restored).

Treatment involves the use of a whole range of drugs

With a progressive and complex course - a complex of medications. Given the specifics of the disease, it is carried out in three directions: elimination of symptoms and prevention of the development of secondary pathologies, prevention of SCD, regulation and maintenance of the neurohumoral system:

• Drugs that restore the rhythm. To eliminate ventricular tachycardia and atrial fibrillation - Amiodarone or Disopyramide. Supplement with inhibitors, diuretics. With more than one risk indicator, SCD is not prescribed.
• Adrenoblockers (Metoprolol, Nadolol, Propranolol). Used for obstructive or non-obstructive form. Effective in 30-60%. They suppress the activity of the sympathoadrenal system, which reduces the need for oxygen in the heart muscle. Eliminate symptoms and cause regression of hypertrophy. The approximate dose is 20 mg three times a day, varies depending on the dynamics of pressure, frequency of contractions and manifestations of insufficiency. Anticoagulants are prescribed to prevent thrombus formation.

Metoprolol helps to reduce the manifestations of hypertrophy

• Calcium channel blockers (Verapamil, Disopyramide). Suppress hypertrophy and eliminate symptoms. The functions are similar to the previous group, the effectiveness was confirmed in 60-80% of cases. Dangerous gradient increase. The method of application is the same. Under the supervision of a doctor, the dose is increased.

Any treatment is carried out strictly in conditions medical institution and under constant supervision.

This video will tell about the features of hypertrophic cardiomyopathy and its dangers: