Cardiomyopathy

Cardiomyopathy is a group of diseases associated with functional, mechanical or electrical problems which lead to ventricular hypertrophy or enhancement of cardiac muscle.

 

This is due to a variety of reasons, most of which are genetic. Cardiomyopathy is defined as a distinct disease which has arisen as a result of no other causes of heart muscle damage, for example ischemic heart disease, valvular heart disease or high blood pressure.

 

Clinical symptoms include shortness of breath and fatigue. In view of the different genes and hereditary diseases that cause cardiomyopathy and hypertrophic cardiomyopathy, a very important clinical parameters such as:

 

  • the cases of sudden cardiac death in the family;
  • muscular dystrophy;
  • a family history of pacemaker implantation.

 

 

What are the types of cardiomyopathy?

 

Dilated cardiomyopathy

 

It is the most common form of the disease. It develops slowly over many years, while cardiac contractility decreases with time. The reason for this is not clear, but there are cases when the disease is caused by genetic factors, viral infections or autoimmune diseases, toxins.

 

By decreasing cardiac contractility cardiac occupancy pressure increases and, consequently, the heart enlarges. As a result of increased heart pressure, pulmonary edema occurs, which is accompanied by shortness of breath. This is a serious disease, which in many cases can lead to death.

 

Two-thirds of cases of cardiomyopathy due to coronary artery disease enlarged. Another third is due to non-ischemic conditions, such as uncontrolled high blood pressure, the effects of alcohol, idiopathic causes or infection.

 

Hypertrophic cardiomyopathy

 

It is a genetic disease that can occur at any age. It is caused by the components responsible for the mechanism of muscle contraction of the heart (eg, damage to the cell-type chain myosin). As a result, this enlarging the heart (cardiac hypertrophy). Left ventricular hypertrophy leads to asymmetries of the interventricular septum, and usually entails blocking of the aortic valve. Symptom of the disease is atrial fibrillation.

 

Restrictive cardiomyopathy

 

It is a rare disease that is characterized by a decrease in ventricular response, causing it to be filled with blood are not fully. The disease is usually caused by other diseases associated with inflammation of the heart muscle. For example, sarcoidosis and amyloidosis.

 

 

The main symptoms of  cardiomyopathy

 

Cardiomyopathy has various signs and symptoms:

 

  • background ventricular arrhythmias, especially in young patients with no symptoms before the age of 35 (including athletes);
  • cardiac insufficiency and angina pectoris: shortness of breath, often accompanied by angina, can lead to progressive heart failure even with normal sinus rhythm and the systolic function;
  • atrial fibrillation (atrial fibrillation) or chronic atrial attack, worsening heart failure, and poses a risk of thromboembolism and stroke;
  • blockage of the left ventricle;
  • diastolic dysfunction;
  • concentric hypertrophy;
  • ischemia of the heart muscle (myocardium);
  • autonomic dysfunction (autonomic dysfunction);
  • mitral valve insufficiency.

 

Diagnosis of cardiomyopathy

 

In addition to a medical examination by a cardiologist, the diagnosis is made by echocardiography and CMR.

 

Patients diagnosed with hypertrophic cardiomyopathy should undergo genetic counseling, which also recommended for first-degree relatives. Genetic tests are an important tool to confirm the diagnosis and to identify relatives carrying the gene.

Also performed ECG and Holter monitoring heart.

 

In some cases, our experts can also recommend pass the stress-test to assess functional capacity, risk assessment and response to treatment.

 

Patients who complain of pain in the chest, can be tested for coronary stenosis (as indicated by the doctor). Patients with high risk factors for coronary artery disease is often recommended diagnostic coronary angiography (cardiac catheterization).Patients with a low chance of obstructive coronary artery disease may be recommended CTA (CT angiography). Echocardiography in such cases is not enough.

 

Family members – carriers of the disease should undergo periodic clinical examination, ECG and echocardiography.

 

Tests should be done every 12-18 months, aged 12-18 years. The rating is up to 12 years of age is required only in cases of strict family history for children involved in sports, or in the case of the onset of clinical symptoms.

 

Treatment of cardiomyopathy

 

Asymptomatic cardiomyopathy

 

Patients and carriers of the disease in order to reduce risk factors for coronary heart disease need:

 

  • to stop smoking,
  • to control blood pressure,
  • to carry out prevention of diabetes mellitus,
  • to perform hyperlipidemia analysis,
  • to control a healthy weight and diet, as well as maintain a healthy lifestyle.

Asymptomatic patients are also recommended regular exercise at low intensity. In some cases, our doctors prescribe medications to maintain hydration and to avoid situations which might cause the enlarging of peripheral blood vessels.

 

Medication for symptomatic cardiomyopathy

 

Drug therapy is prescribed by an experienced cardiologist, individually for each patient, based on carefully conducted survey. A new generation of drugs, their correct dosage and compliance rate prescribed by experienced specialist, allow our patients to get rid of the symptoms as well as prevent the development of disease and possible complications.

 

Invasive procedures for symptomatic cardiomyopathy

 

In some instances, the mini-invasive procedures for reducing the thickness of the interventricular septum, and hence alleviate the symptoms. They are generally recommended for patients with a gradient greater than 50 mm Hg (at rest or under stress), which did not react to an optimal pharmacological therapy.

 

This procedure is performed by catheterization (ablation). During her failure occurs pathological cardiac muscle portion. Procedure is performed by an experienced cardiac surgeon, in closed sertce, through the vein of the patient, and does not require a long hospital stay and recovery.

 

Some patients are recommended mitral valve replacement with a view the decrease of the pressure gradient in the left ventricle.

 

As a rule, patients with reduced left ventricular function are treated similar to patients with heart failure. Patients with heart failure class 3-4, no obstruction of the left ventricle, may be recommended implantation of a pacemaker, if there is no contraindication.

 

Patients with end-stage heart failure may be candidates for a heart transplant.

 

Prevention of sudden cardiac death

 

All patients with hypertrophic cardiomyopathy are evaluated the risk of sudden cardiac death. Components of risk include:

 

  • history of ventricular fibrillation,
  • ventricular tachycardia or sudden death in the family,
  • the transition loss of consciousness without explanation,
  • thickening of the left ventricle of more than 30 mm.,
  • abnormal blood pressure response during physical exertion.
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