Hypertrophic cardiomyopathy is characterized by a significant thickening of the myocardium of one of the ventricles, which somewhat reduces its density. The disease can be completely asymptomatic, however, despite this, therapy must be approached with all responsibility, since the disease can lead to sudden death.
Therapy can be conservative, and in difficult cases surgery is indicated.
Features of the disease
According to statistics, hypertrophic cardiomyopathy develops in about 1% of people. Basically, this disease occurs in men aged 30-50 years. In some cases, pathology develops coronary atherosclerosis, and the condition may be complicated by infective endocarditis. Very rarely, hypertrophic cardiomyopathy occurs in children.
In the development of this disease, an increase in cardiac fibers plays an important role. Two pathological mechanisms underlie. ATas a result of their flow, an insufficient amount of blood enters the ventricles of the heart, which is explained by poor elasticity of the myocardium, due to which the pressure rises very quickly. The heart of the patient loses the ability to relax normally.
Obstructive hypertrophic cardiomyopathy is a disease in which the wall between the ventricles is thickened and the movement of the mitral valve leaflets is impaired. In this case, left atrial disorders develop, and a little later - pulmonary hypertension.
Quite often, obstructive hypertrophic cardiomyopathy is complicated by coronary heart disease. During the pathological process, muscle fibers acquire a fibrous structure after some time, and the cardiac arteries are somewhat modified. This disease is mainly hereditary, but it can also develop due to a sharp mutation of genes.
Forms and classification
There are several classifications of hypertrophic cardiomyopathy. Since the heart muscle can increase in different ways, doctors distinguish a symmetrical and asymmetric form of the disease. Symmetrical is characterized by the fact that the walls of the left ventricle thicken equally. In some cases, there may be a simultaneous increase in the thickness of the right ventricle.
Asymmetrical shape is quite common. Basically, there is a thickening at the bottom, in the middle or on top of the interventricular septum. At the same time, it thickens several times. The disease can prevent the passage of blood into the aorta. Based on this factor, 2 forms are distinguishedhypertrophic cardiomyopathy: obstructive and non-obstructive.
The degree of thickening can be very different. Depending on this, several stages of the course of the disease are distinguished. The first stage is characterized by a slight increase in pressure, and it also proceeds without severe symptoms, so the patient is practically not bothered by anything.
At stage 2, a person experiences slight discomfort during physical exertion. At stage 3, the patient's symptoms become more pronounced, and there are signs of angina pectoris, as well as shortness of breath, even at rest. In the last stage, very serious circulatory disorders occur, and the likelihood of sudden death increases.
Doctors distinguish primary and secondary forms of the disease. The reasons for the emergence of the primary form are still not fully understood. Basically, it occurs as a result of gene mutations, which can be hereditary or acquired. The secondary form mainly occurs in older people with high blood pressure and congenital changes in the structure of the heart muscle.
Causes of occurrence
The most common provoking factor is heredity. Pathology is often genetic in nature, as it has an autosomal type of inheritance. As a result of this, pathological growth of some fibers of the heart muscle occurs. It has been established that compaction of the left ventricle is completely unrelated to heart muscle defects, ischemic disease, hypertension, and other pathologies that can causesimilar changes. Other reasons include:
- spontaneous mutation of genes;
- long-term hypertension;
- old age.
In the presence of a persistent gene mutation, which is in no way associated with a genetic predisposition, there is a deterioration in protein synthesis. Such violations may be associated with harmful working conditions, smoking, pregnancy, and some infections.
Sustained hypertension can provoke secondary cardiomyopathy. It develops in the elderly and proceeds with pathological changes in the structure of the heart muscle.
Main symptoms
Symptoms of hypertrophic cardiomyopathy largely depend on the form of the disease. The non-obstructive form practically does not cause any discomfort to the patient, since the blood flow is not disturbed. With an obstructive condition, the patient is accompanied by shortness of breath, impaired blood flow from the ventricles, as well as an irregular pulse, which mainly occurs during physical exertion.
Hypertrophic cardiomyopathy is characterized by the following symptoms:
- shortness of breath;
- fainting;
- dizziness;
- extrasystole;
- arterial hypotension;
- paroxysmal tachycardia;
- puffiness of the lungs;
- cardiac asthma.
Loss of consciousness and dizziness are associated with poor circulation, which occurs as a result of a decrease in the volume of blood that enters the aorta. Symptoms increase when a person takes an upright position, after doing hard work, as well as eating food.
Painful sensations in the chest are mostly pressing in nature and are felt behind the sternum. This occurs as a result of an increase in the mass of the heart muscle, which begins to experience a greater need for oxygen, while the volume of blood in the body's own vessels decreases significantly.
Another sign of the course of the disease is cardiac death, which manifests itself in the form of loss of consciousness approximately 1 hour after the onset of the first signs of pathology.
Hypertrophic cardiomyopathy with obstruction is quite complex and is accompanied by pulmonary edema and cardiac asthma. However, despite the existing symptoms, quite often the only manifestation of such a violation is the sudden death of the patient.
Diagnostics
Diagnosis of hypertrophic cardiomyopathy begins with the collection of patient complaints and the study of his family history. The doctor finds out exactly when the first symptoms appeared, what the patient and his relatives were previously ill with. For the initial diagnosis, the doctor conducts a physical examination. Initially, he evaluates the shade of the skin, since during the course of the disease their cyanosis may be observed.
Then he performs tapping, which allows you to determine how much the size of the heart muscle is enlarged on the left side. Then you need to listen to the noise over the aorta. Similar violationobserved if the cavity of the ventricle is significantly narrowed. To confirm the diagnosis of hypertrophic cardiomyopathy, such types of research are prescribed as:
- urine and blood test;
- biochemical analysis;
- expanded coagulogram;
- electrocardiogram;
- chest x-ray;
- tomography;
- phonocardiogram.
If the data of the conducted studies did not help to make a diagnosis, then the patient may additionally be prescribed cardiac muscle catheterization and endocardial biopsy. In addition, consultation with a general practitioner and a cardiac surgeon is required.
Features of treatment
Treatment of hypertrophic cardiomyopathy is carried out with the help of medications, folk remedies, and also through surgery. Therapy with folk remedies does not give a special result, only slightly improves the patient's well-being. For treatment, such remedies as motherwort, viburnum, St. John's wort, calendula are used.
Drug therapy is indicated only in the presence of severe symptoms of the disease or with the threat of sudden cardiac death. Calcium channel antagonists, as well as beta-blockers, are introduced into the therapy regimen. For severe heart rhythm disturbances, antiarrhythmic drugs are prescribed. In case of heart failure and stagnation in the process of blood circulation, the use of diuretics, cardiac glycosides, as well as beta-blockers is indicated.
In severe cases, surgicalan intervention that will help normalize the patient's well-being and prevent the development of complications.
Conservative Therapy
Drug treatment of hypertrophic cardiomyopathy is prescribed to maintain the normal state of he alth of the patient and his performance. With a moderate course of the disease and not too severe symptoms, patients are prescribed beta-blockers or calcium channel blockers, which help to relax the heart muscle and reduce its stiffness. Long-term use of such drugs can reduce the severity of left ventricular hypertrophy and prevent the formation of atrial fibrillation.
Basically, doctors prescribe non-selective beta-blockers, in particular, Anaprilin, Obzidan, Inderal. In some cases, the use of selective drugs such as Metoprolol and Atenolol is indicated. The choice of drug depends largely on the individual characteristics of patients.
During therapy, doctors may prescribe calcium antagonists. These drugs affect the concentration of the element in the systemic coronary arteries. When using such drugs, diastolic relaxation of the left ventricles can be normalized, as well as reduce myocardial contractility. All drugs used have pronounced antiarrhythmic and antianginal properties. A good result is shown by means such as Finoptin and Isoptin. Also, the doctor may prescribe "Kardizem" and "Kardil", it all depends onfeatures of the course of the disease.
The preparations "Ritmilen" and "Amiodarone" are recommended for the treatment of patients who are at high risk of sudden death. Such drugs have a pronounced antiarrhythmic effect.
If the patient has heart failure, the doctor may prescribe diuretics. Recently, therapy has been carried out with the help of ACE inhibitors, for example, Enalapril.
Surgery
After diagnosing hypertrophic cardiomyopathy, treatment should be prescribed immediately, since such a disease can lead to dangerous complications, as well as sudden death of the patient. In modern medicine, several types of surgical intervention are practiced:
- ethanol ablation;
- resynchronization therapy;
- myotomy;
- implantation of a cardioverter-defibrillator.
Ethanol ablation is performed by injecting a solution of medical alcohol into the thickened septum of the heart. A similar procedure is carried out under the strict supervision of a doctor through a small puncture of the chest. Under the influence of concentrated alcohol, the cells die, as a result of which the walls located between the ventricles become thinner.
Myotomy is performed on an open heart and involves the removal of the internal septum. The essence of resynchronization therapy is to restore the disturbed conduction of the heart muscle. The surgeon performs implantationan electrical stimulator that helps normalize blood circulation and prevent complications.
Implantation of a cardioverter-defibrillator helps to fix the cardiac cardiogram in case of serious heart rhythm disturbance, and then it sends a pulse to the heart, restoring its normal rhythm. The technique of surgical intervention depends on each specific case.
Lifestyle
With hypertrophic cardiomyopathy, doctors' recommendations also apply to lifestyle. Diet recommended. Daily loads are not limited, however, there is a ban on exercise even after drug therapy or surgery. It is believed that after 30 years the risk of sudden death is negligible, which is why, in the absence of aggravating factors, you can gradually move on to moderate physical activity.
Be sure to give up bad habits. You should avoid food that provokes an increase in the level of cholesterol in the blood, as well as a violation of the outflow of lymph and blood. It is forbidden to eat fatty foods, as well as spicy and s alty foods.
Possible Complications
With hypertrophic cardiomyopathy, clinical guidelines must be followed. Otherwise, various dangerous complications may arise. The most common of these is a deterioration in heart rate. Arrhythmia is observed in most patients. If timely treatment is not carried out, it can lead to heart failure. In addition, there are suchcomplications like:
- infective endocarditis;
- vascular thromboembolism;
- chronic heart failure.
Besides this, other complications may develop, which is associated with impaired circulation of various organs and systems. As a result of the development of diseases, many patients die suddenly. The risk of death largely depends on the age of the patient and many other factors. Often this happens in childhood and young age.
Forecast and prevention
In some cases, there is a regression of the disease without treatment, however, if therapy is not carried out, death may occur. It should be noted that in hypertrophic cardiomyopathy, the average life expectancy is slightly higher than in the dilated type of the disease. In addition, mortality depends on the age of the patient. For younger patients, the prognosis is worse if surgery is not performed in a timely manner.
There is no specific prevention of such a disease. However, to prevent the risk of developing pathology, it is necessary to conduct a periodic examination if relatives have cardiomyopathy. This allows you to diagnose the disease in the initial stages. In addition, a low-s alt diet is recommended.
