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Restrictive cardiomyopathy

Restrictive cardiomyopathy (RCMP) is known in two versions of Fanee, considered as two distinct pathological processes) - endocardial fibrosis and endomyocardial fibroelastosis * eFflera. The pathomorphological picture of the two diseases is a little different.


It is characterized and characterized by a sharp thickening of the endocardium in combination with ventricular myocardial hypertrophy, the cavities of which can be expanded. reny or reduced. Usually both ventricles are involved in the pathological process, less often one. The most typical is a change in the right ventricle with involvement of the papillary muscles and tendon chords with progressive obliteration of the ventricular cavity.

PCMP (as a syndrome) can also be observed in case of diseases leading to impaired diastolic relaxation (the so-called accumulation diseases — amyloidosis, hemochromatosis, glycogenosis). In these diseases, pathological substances are accumulated in the myocardium, causing a violation of myocardial relaxation.

Pathogenesis. In RCMP, a violation of the diastolic filling of the ventricles occurs in the form of shortening the time of isometric relaxation of the ventricles and a decrease in the late filling of the ventricles (during atrial systole). These disorders, as well as the often developing tricuspid valve insufficiency, cause an increase in heart failure (often with small heart sizes).

Clinical picture. Manifestations of the disease are extremely polymorphic, they are determined by the symptoms of circulatory disorders in a small or large circle (depending on the preferential damage to the right or left ventricle).

At the first stage of the diagnostic search, it is not always possible to obtain the necessary information for making a diagnosis, since complaints are either absent or due to stagnation in the small or major circulation.

At stage II, symptoms of congestive heart failure of varying severity can be detected. Of greater importance is the detection of an increase in the heart, a soft late systolic noise and a loud early III tone (due to the rapid filling of the ventricles during diastole). Kussmaul's symptom may be detected - an increase in the swelling of the jugular veins during the deep breath phase.

At the third stage of the diagnostic search, there are usually no signs of an enlarged heart during X-ray examination. On the ECG, nonspecific changes of the T wave, blockade of the left (less often - right) leg of the bundle of His are noted. An echocardiographic study shows a decrease in the cavity of the left ventricle, a shortening of the time of isovolemic relaxation (signs of impaired diastolic function of the left ventricle). With Doppler echocardiography, an increase in the peak of early filling, a decrease in the peak of late atrial filling of the ventricles and an increase in the ratio of early filling to late is noted. In addition, rapid movement of the anterior mitral valve of the mitral valve during diastole and rapid early movement of the posterior wall of the left ventricle outside are detected.

In the study of parameters of central hemodynamics, an increased filling pressure in both ventricles is determined, with the final pressure in the left one exceeding that in the right ventricle.
During ventriculography, the left ventricle, or both ventricles, are observed to contract tightly, the smooth contours of their walls, sometimes with a filling defect in the area of ​​the apex, reflecting its obliteration. In some cases, depressions in the papillary muscles are determined. Signs of insufficiency of mitral or tricuspid valves are noted.

With endomiocardial fibroelastosis of Leffler, severe eosinophilia and bronchospastic syndrome are possible, which, in combination with


An abnormal increase in the size of the heart and symptoms of heart failure helps to make the correct diagnosis.

Diagnostics. Recognizing the RCMP is extremely difficult. It is possible to speak confidently about this pathology with the exception of, first of all, kojastitivnogo pericarditis with signs of circulatory disorders in a large circle, as well as heart defects (mitral-tricuspid ass). The assumption of rcmp may occur when a moderate increase in the heart (sometimes normal heart size) is detected in combination with signs of heart failure that cannot be explained by any other reasons. From this it follows that it is necessary to conduct a number of additional research methods. Early stages of the disease can be detected by probing the heart and detecting increased end-diastolic pressure in the left ventricle. Currently, with the help of Doppler, it is possible to identify violations that occur during the diastole period. An increase in the peak of the early filling, a decrease in the peak of the late filling, an increase in the ratio of the early filling to the late is found. Help in establishing the diagnosis also provides angiocardiography.

Treatment is usually symptomatic and is aimed at reducing stagnation in the small and large circulation. Diuretics (hydrochlorothiazide at a dose of 50 mg / day), vasodilators (isosorbide dinitrate and isosorbide mononitrate 20–60 mg / day), indirect anticoagulants are used in small doses. Diuretics and peripheral vasodilators in high doses can worsen the condition. Cardiac glycosides are not used.

Surgical treatment of fibroplastic endocarditis consists in excision of the thickened endocardium, release of the tendon chords and tissue of the valves. In case of severe damage to the valves, they are made prosthetics.

Forecast. Mortality within 2 years is 35-50%. Restrictive cardiomyopathy may be complicated by thromboembolism, arrhythmias, progression of heart failure.
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Restrictive cardiomyopathy

    Definition Restrictive cardiomyopathy is an infiltrative or fibrous myocardial lesion, which is characterized by rigid, uncompliant ventricular walls, decreased filling and diastolic volume of one or both ventricles with normal or almost unchanged systolic function and wall thickness. The basis of the disease is common interstitial fibrosis.
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