Restrictive cardiomyopathy is a disease caused by a change of the heart - and the endocardium is characterized by impaired diastolic function of the heart.
The inner membrane of the heart (endocardium) consists of connective tissue. It becomes thicker and denser, with a modified endocardium can not be stretched into a phase of diastole (relaxation). This disrupts the normal blood-filled ventricles and in the end there is circulatory failure. In addition, the heart muscle can not fully relax in the diastolic phase, and this violates its blood supply and nutrition.
Restrictive cardiomyopathy - the most rare form of cardiomyopathy. It may be a separate disease, and one of the components of various diseases (amyloidosis, hemochromatosis, endomyocardial fibrosis, Fabry disease, a disease Leffler, scleroderma).
There are two forms of restrictive cardiomyopathy:
Obliterans, when the inner membrane of the heart and laid it under the abnormal proteins. Then the seal and endocardial thickening (fibrosis), on the walls of the heart cavities formed thrombi and obliteration occurs (reduction, narrowing) of the internal cavities of the heart. Can marvel at the right, left or both halves of the heart.
Diffuse infiltration of the myocardium. At the same abnormal substances are deposited uniformly throughout the heart muscle.
The origin of restrictive cardiomyopathy remains unclear, as many of those diseases which are accompanied by the emergence of this type of cardiomyopathy. It is assumed viral or hereditary in origin. But the proven theories yet.
In early disease, patients may complain of weakness, reduced exercise tolerance, shortness of breath. Pain in the heart or chest is rare. In the future, there is gradual heart failure, depending on where endocardial lesions on the left or right ventricular type. Often the disease is detected only when the patient is having symptoms of heart failure: swelling in the legs, ascites (accumulation of fluid in the abdomen), cyanosis of the face, swelling of the neck veins.
Auscultation of heart sounds are heard muffled, sometimes systolic murmur. An electrocardiogram revealed a variety of arrhythmias, low cardiac complexes teeth. On the radiograph usually increase the right atrium and right ventricle, changes in the lungs. On echocardiogram define a thickening of the heart, reducing the volume of the cavities of the ventricles, abnormal movement of the interventricular septum, often valvular disorders. In angiocardiography (research with contrast media) exhibit a decrease in size, uneven contours of the ventricles of the heart, decreased cardiac output. Sometimes endocardial biopsy - a study under the microscope a small piece of the inner lining of the heart.
Treatment of restrictive cardiomyopathy.
Effective treatment of this disease has yet been found. Symptomatic treatment of heart failure occurs when small doses of diuretics and digoxin. The other drugs are being used with great caution. Heart transplantation is most often ineffective, since some time in the transplanted heart is developing the same process. The condition of patients is usually mild and severe. 70% of patients die within 5 years.