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, the treatment should be approached with all responsibility, since the disease can lead to sudden death.
Therapy can be conservative, and in complex cases, surgical intervention is indicated.
Features of the disease
According to statistics, hypertrophic cardiomyopathy develops in approximately 1% of people. Basically, this disease occurs in men aged 30-50 years. In some cases, with pathology, coronary atherosclerosis develops, and the condition can be complicated by infectious endocarditis. Very rarely, hypertrophic cardiomyopathy occurs in children.
In the development of this disease, an important role is played by an increase in heart fibers. Two pathological mechanisms are the cornerstone. As a result of their flow, insufficient blood flows into the ventricles of the heart, which is explained by the poor elasticity of the myocardium, due to which the pressure rises very quickly. The patient’s heart loses its 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 cusps is impaired. In this case, violations of the left atrium develop, and somewhat later - pulmonary hypertension.
Quite often, obstructive hypertrophic cardiomyopathy is complicated by coronary heart disease. In the course of the pathological process, muscle fibers after some time acquire a fibrous structure, 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 symmetric and asymmetric form of the disease. Symmetric is characterized by the fact that the walls of the left ventricle thicken equally. In some cases, a simultaneous increase in the thickness of the right ventricle may be observed.
The asymmetric form is quite common. Basically, there is a thickening below, in the middle or on top of the interventricular septum. At the same time, it thickens several times. The disease can interfere with the passage of blood into the aorta. Based on this factor, 2 forms of hypertrophic cardiomyopathy are distinguished: 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 worried about anything.
At stage 2, a person has slight discomfort during physical exertion. At the 3rd stage, the patient's symptoms become more pronounced, and there are signs of angina pectoris, as well as shortness of breath even at rest. At the last stage, very serious circulatory disorders occur, and the likelihood of sudden death increases.
Doctors distinguish the primary and secondary form of the disease. The causes of the appearance of the primary form are still not fully understood. Basically, it arises as a result of gene mutations that 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 triggering 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, coronary artery disease, hypertension, as well as other pathologies in which such changes can occur. Among other reasons, the following can be distinguished:
- spontaneous mutation of genes;
- prolonged hypertension;
- elderly age.
In the presence of a persistent gene mutation, which is in no way associated with a genetic predisposition, protein synthesis is impaired. Such violations can be associated with harmful working conditions, smoking, pregnancy, and also some infections.
Secondary cardiomyopathy can cause persistent hypertension. It develops in elderly people and proceeds with pathological changes in the structure of the heart muscle.
Main symptoms
Symptoms of hypertrophic cardiomyopathy are largely dependent on the form of the disease. The nonobstructive form practically does not cause the patient any discomfort, since the blood flow is not impaired. In obstructive condition, the patient is accompanied by shortness of breath, disturbed blood flow from the ventricles, as well as an irregular heartbeat, which mainly happens when physical activity is performed.
Hypertrophic cardiomyopathy is characterized by the appearance of such symptoms:
- dyspnea;
- fainting state;
- dizziness;
- extrasystole;
- arterial hypotension;
- paroxysmal tachycardia;
- pulmonary edema;
- cardiac asthma.
Loss of consciousness and dizziness are associated with poor blood 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 oppressive and felt behind the sternum. This occurs as a result of an increase in the mass of the heart muscle, which begins to experience a great need for oxygen, while the volume of blood in its own vessels of this organ is significantly reduced.
Another sign of the course of the disease is cardiac death, which manifests itself in the form of loss of consciousness about 1 hour after the onset of the first signs of pathology.
Hypertrophic cardiomyopathy with obstruction is quite complicated 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 collecting patient complaints and examining his family history. The doctor finds out exactly when the first symptoms appeared, the earlier the patient and his relatives were sick. To make an initial diagnosis, the doctor performs a physical examination. Initially, he assesses the shade of the skin, since with the course of the disease their cyanosis can be observed.
Then she taps, which allows to determine how much the size of the heart muscle on the left side is increased. Then listening to the sounds above the aorta is required. A similar violation is observed if the ventricular cavity narrows significantly. To confirm the diagnosis of hypertrophic cardiomyopathy, types of studies are prescribed as:
- analysis of urine and blood;
- biochemical analysis;
- detailed coagulogram;
- electrocardiogram;
- chest x-ray;
- tomography;
- phonocardiogram.
If the data from the studies did not help to make a diagnosis, then the patient can additionally be prescribed catheterization of the heart muscle and endocardial biopsy. In addition, consultation with a physician and a cardiac surgeon is required.
Treatment features
Treatment of hypertrophic cardiomyopathy is carried out with the help of medications, folk remedies, as well as by surgical intervention. Therapy with folk remedies does not give a special result, it only slightly improves the patient's well-being. For treatment use such drugs as motherwort, viburnum, St. John's wort, calendula.
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 treatment regimen. In case of serious cardiac arrhythmias, antiarrhythmic drugs are prescribed. In the case of heart failure and congestion in the blood circulation, the use of diuretics, cardiac glycosides, as well as beta-blockers is indicated.
In severe cases, surgical intervention is indicated 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 patient's normal health and his performance. With a moderate course of the disease and not too pronounced symptoms, patients are prescribed beta-blockers or calcium channel blockers, which help relax the heart muscle and reduce its rigidity. The prolonged 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 the patients.
During the treatment, doctors may prescribe calcium antagonists. These drugs affect the concentration of an element in the systemic coronary arteries. With the use of such drugs, it is possible to normalize diastolic relaxation of the left ventricles, as well as reduce myocardial contractility. All drugs used have pronounced antiarrhythmic and antianginal properties. A good result is shown by such funds as Finoptin and Isoptin. Also, the doctor can prescribe "Cardizem" and "Cardil", it all depends on the particular course of the disease.
The drugs "Rhythmilen" and "Amiodarone" are recommended for the treatment of patients who have a high probability of sudden death. Such drugs have a pronounced antiarrhythmic effect.
If the patient has heart failure, then the doctor may prescribe diuretics. Recently, therapy has been carried out using ACE inhibitors, for example, Enalapril.
Surgical intervention
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 carried out by introducing 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, 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 an internal septum. The essence of resynchronization therapy is to restore impaired conduction of the heart muscle. The surgeon performs the implantation of an electric stimulator, which helps to normalize blood circulation and prevent the occurrence of complications.
The implantation of a cardioverter-defibrillator helps to fix the cardiogram in case of a serious violation of the heart rhythm, and then it sends an impulse to the heart, restoring its normal rhythm. The method of surgical intervention depends on each specific case.
Lifestyle
With hypertrophic cardiomyopathy, doctors' recommendations also relate to lifestyle. Diet recommended. Daily workloads do not limit, however, there is a ban on physical 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 exertion.
Be sure to abandon bad habits. You should avoid food that provokes an increase in blood cholesterol, as well as a violation of the outflow of lymph and blood. It is forbidden to eat fatty foods, as well as spicy and salty foods.
Possible complications
With hypertrophic cardiomyopathy, clinical recommendations must be followed. Otherwise, various dangerous complications may occur. The most common of these is heart rate deterioration. Arrhythmia is observed in most patients. If treatment is not done in a timely manner, it can lead to heart failure. In addition, we can distinguish such complications as:
- infectious endocarditis;
- vascular thromboembolism;
- chronic heart failure.
In addition, other complications may develop, which is associated with circulatory disorders of various organs and systems. As a result of the development of diseases, many patients suddenly die. The risk of death 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 is worth noting that with hypertrophic cardiomyopathy, the average life expectancy is slightly higher than with the dilated type of the disease. In addition, mortality depends on the age of the patient. For young patients, the prognosis is worse if surgery is not performed in a timely manner.
Specific prevention of such a disease does not exist. However, in order to prevent the risk of developing pathology, a periodic examination should be carried out if cardiomyopathy is observed in relatives. This allows you to diagnose the disease in the initial stages. In addition, a low-salt diet is recommended.