Restrictive cardiomyopathy (from the Latin wordrestrictio «constraint") - a disease characterized by a pronounced decrease in distensibility of the myocardium, due to various reasons, and accompanied by a lack of blood filling the heart with the subsequent development of chronic heart failure.
The causes of the disease
The only proven cause ofPrimary restrictive cardiomyopathy is the so-called hypereosinophilic syndrome, common in tropical countries. Eosinophils - blood cells involved in the development of inflammatory reactions, especially allergic nature.
In hypereosinophilic syndrome (diseaseLeffler) endocardial inflammation happens - the inner lining of the heart. Over time, this inflammation terminates significant compaction endocardial and rude with his spayaniem adjacent myocardium, which leads to a drastic reduction of the heart muscle extensibility. In most cases, the origin of restrictive cardiomyopathy is secondary, that is due to other causes. Amyloidosis - a disease related to metabolic disorders in the body, particularly protein metabolism. When amyloidosis is formed and deposited in the tissues of various organs in a large number of abnormal protein (amyloid). With the defeat of the heart, the protein literally "impregnates" the myocardium, causing a significant reduction in its contractility and extensibility. Hemochromatosis - a rare disease, which interferes with iron metabolism in the body. The iron content in the blood is increased in hemochromatosis, an excess is deposited in many organs and tissues, including the myocardium, causing a decrease in its elongation. Sarcoidosis - a disease of unknown origin, characterized by the formation in the organs and tissues of specific cell clusters (granulomas). The most common sarcoidosis lung, liver, spleen and lymph nodes.
The mechanism of development of restrictive cardiomyopathy
Restriction stretchability leads to infarctionlowering blood filling the ventricular and intraventricular pressure increase significantly. With restrictive cardiomyopathy organism compensatory abilities reduced. Therefore, after some time there is stagnation in the small (pulmonary) circulation and decreased cardiac pump function. Gradually develop persistent leg swelling, hepatomegaly (enlarged liver), and abdominal dropsy (ascites) due to fluid retention in the body due to decreased cardiac pump function.
If the seal extends to the endocardialvalves (mitral and tricuspid), the condition is exacerbated by the development of their disease. In the cavities of the heart due to their reduced contractility and extensibility can form clots. Distribution of fragments of blood clots from the bloodstream (embolism) can cause heart attacks internal organs and occlusion of large vessels. With restrictive cardiomyopathy are also often observed different types of arrhythmias.
The main manifestations of the disease
Patients with restrictive cardiomyopathy typicallyThey complain of shortness of breath, initially during exercise (jogging, brisk walking, etc.), as well as the disease progresses and alone. Due to the reduction of the pumping function of the heart is a typical complaint of fatigue and poor tolerance of any physical activity. Then join leg swelling, enlarged liver and abdominal dropsy. Occasionally patients complain of irregular heartbeat. With the development of persistent blockages may occur fainting.
Methods of diagnosis of restrictive cardiomyopathy
Echocardiography is oneof the most informative methods for diagnosis of the disease. It reveals thickening of the endocardium, the changing nature of the filling of the heart ventricles, decreased cardiac pump function and the presence of the valve failure. Magnetic resonance imaging - a method that allows using a special computer processing information about the anatomy of the heart, to identify abnormal inclusions in the myocardium and measure the thickness of the endocardium. Probing the heart is usually performed to measure the pressure in the cavities of the heart and identify specific changes for this disease. Conducting this research is necessary in cases of dispute, in particular, to exclude the outer lining of the heart disease (pericarditis) - constrictive pericarditis.
In vivo myocardial biopsy and usually endocardialIt is performed simultaneously with the sensing of the heart. This method has the most informative to clarify the nature of the disease and further treatment. Since the defeat of the myocardium in some embodiments, restrictive cardiomyopathy, such as sarcoidosis, does not cover the entire myocardium, the biopsy may not bring the desired success. This is explained by the fact that when taking a biopsy the doctor technically can not see directly the myocardium. Therefore, the modified portion of the myocardium can not get to the number of samples of heart tissue, selected for further microscopic examination. In extremely rare cases, when the above diagnostic methods do not allow accurate diagnosis, conduct a direct audit of the pericardium on the operating table. All patients restrictive cardiomyopathy is necessary to conduct comprehensive clinical, biochemical, and further investigation to identify the causes extracardiac diseases, such as amyloidosis and hemochromatosis.
Principles of treatment of restrictive cardiomyopathy
Treatment of the disease is quite difficult. In some cases, diuretics (aldactone, in particular) can be used to remove excess fluid in the body. Most of the drugs commonly used to treat heart failure, are not effective. This is due to the fact that due to the nature of the disease can not achieve a significant improvement of myocardial extensibility (sometimes prescribed for this purpose ions of calcium antagonists). To eliminate persistent conduction abnormalities may require production (implantation) of a permanent pacemaker. Diseases such as sarcoidosis, and hemochromatosis, are subject to self-treatment. Sarcoidosis is treated with hormonal therapy (prednisone and others.), And hemochromatosis - regular bloodletting (to reduce the iron content in the body). amyloidosis infarction Treatment depends on the cause of the amyloidosis. When thrombus formation in the chambers of the heart must be preparatov.Hirurgicheskoe anticoagulant treatment is effective in cases of restrictive cardiomyopathy induced by endocardial lesions. In operation possible excised all changed endocardium. In some cases, when there is a lack of the atrioventricular valves, performed their prosthetics. Some forms of the amyloid lesions infarction treated by means of a heart transplant.
Preventive measures in this diseaselimited. It should be the early detection of potentially avoidable causes of restrictive cardiomyopathy (amyloidosis, sarcoidosis, hemochromatosis, etc.). To achieve these goals may be useful to conduct an annual medical examination of the population.