Restrictive cardiomyopathy is the least frequent form of cardiomyopathy. It implies the presence of virtually non-stretchable walls of the ventricles, which, because of the lesions can not adequately fill the heart chamber with blood. Often affects the left heart, particularly the left ventricle. This cardiomyopathy may be occlusive and obliteriruushih.
The first is subendocardial and fibrosis of the endocardium (inner layer of the heart). The second infiltration of the pathological substance myocardial layer.
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View of the wall of the ventricle with restrictive cardiomyopathy restrictive cardiomyopathies
A restrictive variant of the disease refers to the cardiomyopathy smeshannogo Genesis. This means that the pathology has the properties acquired and genetically determined diseases. There are primary (idiopathic) form of the disease and secondary.
The diagnosis of idiopathic restrictive cardiomyopathy exhibited only in the case if after various examinations and failed to find the cause of the disease. This form of the disease can occur at any age. Studies show that this pathology is often associated (occurs together) with myopathies of skeletal muscle.
Proven genetic component of restrictive cardiomyopathy:
- Mutations of proteins of the sarcomere (one of the components of muscle tissue).
- Family amyloidosis.
- A glycogen storage disease.
- Disease Andreola, paints etc.
Secondary restriction cardiomyopathy cause the following diseases:
- Endomyocardial fibrosis.
- Carcinoid heart disease.
- Hronicheskie toxicity of certain medications.
- Lose heart metastases.
In the presence of primary amyloidosis, the probability of involvement of the myocardium in pathological process. The disease is the deposition in the muscles of the amyloid (the protein-polysaccharide complex), which is a capable to replace the normal cardiomyocytes. The result is rigidity, thickening and compaction of the myocardium. In this case, the dilatation of the cavities is a relatively rare occurrence. When deposits of amyloid in the elements of the conduction system of the heart the disease can lead to various arrhythmias and blockade of conduction. To confirm the diagnosis is echocardiography and endomyocardial biopsy.
In systemic sclerosis restrictive cardiomyopathy develops on the background of constantly progressive fibrosis of the myocardium. This is a violation of diastolic function of the heart muscle. I.e. as in the previous disease broken stretch of wall.
Eosinophilic cardiomyopathy Leffler. Endomyocardial fibrosis
Both of these diseases are associated with an increase in the number of eosinophils in the blood, which eventually cause inflammation of the endocardium. In our latitudes the disease is quite rare. Eosinophilic inflammation occurs mainly in the tissues of the ventricles and atrioventricular valves.
As a result of this lesion is thickening of the endocardium. Along with fibrosis and mural thrombosis it leads to the reduction of the cavity of the heart chamber. Further, due to the dysfunction of the ventricles the blood of them is thrown back into the atrium that leads to hypertrophy (or increase) the latter. After a few years, patients with this disease die of heart failure.
Other diseases of accumulation and infiltrative diseases
Medicine is known for a number of diseases leading to the accumulation in the heart muscle of certain substances that cause restriction cardiomyopathy.
- The disease paints – genetically due to the accumulation inside cells glycolipids.
- Syndrome Herrera – deposition of the elements of the heart of mucopolysaccharides. Affects the valves, the walls of the arteries and the inner layer of the myocardium.
- Gaucher disease is the accumulation of cerebroside due to enzyme deficiency.
- Hemochromatosis – a hereditary disease, manifested in the accumulation of iron in organs, including the myocardium. Usually first affects the liver.
- Systemic sarcoidosis – granulomatous inflammation of the myocardium.
Additional restrictive condition of the body
- Radiation heart disease lose body due to the negative effects of radiation. This condition frequently occurs when the Hodgkin’s disease, when the organs of the mediastinum are exposed to radiation therapy.
- Cardiomyopathy drug-induced – damage to the heart muscle some medical drugs, for example, anticancer.
- Carcinoid heart disease – the occurrence of fibrous plaque on the valve apparatus and the chambers of the right heart. The deposition of metabolites of serotonin when elevated in the blood.
The pathogenesis of restrictive cardiomyopathy the Mechanism of the development of restrictive cardiomyopathy often leads to changes in other tissues and organs. For example, amyloidosis affects only heart muscle, but also cardiac artery. A similar process is observed in hemochromatosis.
The disease paints and sarcoidosis often affect the conductive system of the heart, causing blockade and contributing to the development of various rhythm disorders.
The syndrome Leffler begins his manifestation in the form of inflammation of arteries (arteritis) of eosinophilic origin. Then in the endocardium, valves and chordae of the heart clots. It all progresses to fibrosis.
The consequence of all of the above is the infiltration of the myocardium and/or thickening of the endocardium. These changes do not allow the ventricles to stretch adequately, with the result that gradually develops diastolic dysfunction. In the end, increased venous pressure in the pulmonary circulation, impaired lung function and gas exchange. Parallel to this, the inside of the heart chambers are formed mural thrombi. The danger lies in the fact that these blood clots can at any time to migrate to various organs, causing thrombosis of the arteries. This frequently leads to heart attacks and strokes.
Signs and symptoms of restrictive cardiomyopathy
The symptoms of the disease depend directly on the extent and nature of the lesion of the cardiac elements. Thus, it is possible, as asymptomatic and extremely severe disease with a rapid fatal outcome.
In the early stages possible:
- causeless weakness;
- attacks of nocturnal dyspnea;
- chest pain (only in some forms of amyloidosis).
Signs and symptoms of the disease in the later stages:
- the development of congestive heart failure, signs of liver enlargement and edema in the body, dyspnea in physical load;
- a violation of cardiac conductance and the occurrence of thrombosis;
- the appearance of arrhythmia by type of atrial fibrillation (a-FIB);
- in rare cases, angina and syncope.
Diagnosis of restrictive cardiomyopathy
During examination of the patient may be observed:
- the weakening of the heart sounds when listening to the heart;
- increased heart rate (tachycardia);
- swelling of veins of neck;
- rales in the lungs (especially in the lower divisions);
- the appearance of extraneous noise to the heart.
The main instrumental examinations are needed for the diagnosis of restrictive cardiomyopathy and their interpretation:
- ECG. The electrocardiogram can detect some nonspecific changes of the ST segment. Often found low voltage. Sometimes there are signs of left ventricular hypertrophy or pathological Q-wave, which indicates the transferred earlier myocardial infarction.
- Chest x-ray. The picture shows the heart size is usually unchanged or reduced. The exception is the late stage of amyloidosis and hemochromatosis. In these cases, the cardiac shadow is increased.
- Echocardiography. In this study, systolic function is not impaired. It is possible to detect enlarged atrium. Determined bright echostructure of the myocardium.
- CT (computed tomography) and MRI (magnetic resonance imaging). Research is conducted to explore the changed structure of the pericardium with the iron deposits, or amyloid.
- Catheterization of the myocardium. The need for this research is rare. If kateterizatsii detected high atrial pressure.
- Angiography. Is determined by the regurgitation of the atrioventricular valves (reverse throwing of blood from the cavity of the ventricle into the atrium) and a decrease in the degree of contraction of the heart.
- Biopsy of the myocardium. The study of the structure can be detected fibrosis, thickening of the myocardium and iron deposits or amyloid in it.
- Coronary angiography (study of blood vessels of the heart). Determined by the absence of pathology or deposits in the vessel walls of the above substances.
When examined in the framework of the above instrumental methods it is important to make a differential diagnosis with restrictive pericarditis. In this disease there have been similar changes in the circulation.
Treatment of restrictive cardiomyopathy
Specific drugs used to treat restrictive cardiomyopathy, currently does not exist. This way of therapy is possible only in the case of secondary forms: sarcoidosis, amyloidosis and hemochromatosis. The main medicines for treating a cardiomyopathy aimed at alleviating and eliminating symptoms and preventing possible complications.
- To improve the filling of the ventricles during diastole (relaxation of heart) is used drugs that reduce the heart rate:
- beta-blockers (Inderal, Metoprolol).
- To reduce congestion prescribed drugs that reduce swelling and removing the liquid from the body:
- diuretics (Indapafon, Indapamide, Furosemide).
- With a significant reduction of the contractile function of the ventricles used drugs that increase the force of myocardial contraction:
- cardiac glycosides (Strofantin, Korglikon, Digoxin).
- For the prevention of heart attacks and strokes use of substances that prevent the formation of blood clots:
- antiplatelet agents (Aspirin Cardiomagnyl, Polcard, Aspirin-KARDIO);
- anticoagulants (Heparin).
- In case, if it is of AV-blockade (violation of nervous impulse from the Atria to the ventricles), surgical staging of the triple-EX (pacemaker). Another name – Hebrew (artificial driver rhythm).
- The ineffectiveness of the above treatment sometimes resort to surgical methods:
- prosthetic heart valves;
- transplantation (transplantation) heart.
Prognosis restrictive cardiomyopathy
Forecast, as in dilated cardiomyopathy, adverse. This is because this diagnosis is usually established immediately, but in the later stages. In such cases, showing only a symptomatic treatment.
About 65 % of patients living with this diagnosis for about 5 years. 50% of patients survive after 10 years from the registration of restrictive cardiomyopathy. Almost all patients suffer from cardiac insufficiency of various degrees.
Thus, restrictive cardiomyopathy is a disease associated with impaired distensibility of the cardiac walls. Usually affects one of the ventricles of the heart. The main symptoms of concern to patients – fatigue and shortness of breath. Diagnosis is exposed by using several instrumental methods. The main of them – echocardiography. Symptomatic therapy often ineffective. In severe cases, surgical treatment is indicated