Hospital therapy. Causes of restrictive cardiomyopathy (RCMP) (most important)

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Hospital therapy. Causes of restrictive cardiomyopathy (RCMP) (most important)

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14. Causes of restrictive cardiomyopathy (RCMP)

Restrictive cardiomyopathy (RCMP) - (from the Latin word restrictio - "restriction") - a group of diseases of the myocardium and endocardium, in which, as a result of pronounced fibrosis and loss of elasticity due to various reasons, there is a fixed limitation of filling the ventricles in diastole.

RCMP includes: Lefler's parietal fibroplastic endocarditis (found in countries with a temperate climate, described by W. Loffler et al., 1936) and endomyocardial fibrosis (found in countries of tropical Africa, described by D. Bedford et E. Konstman).

Causes of RCM. Primary RCM is very rare, and the only proven cause of its occurrence is the so-called hypereosinophilic syndrome (Leffler's disease, Leffler's parietal fibroplastic endocarditis). It occurs mainly in men aged 30-40 years. With hypereosinophilic syndrome, inflammation of the endocardium occurs, which over time culminates in significant compaction of the endocardium and its rough adhesion to the adjacent myocardium, which leads to a sharp decrease in the extensibility of the heart muscle. Lefler's syndrome is also characterized by persistent eosinophilia for 6 months or more (1500 eosinophils per 1 mm3), damage to internal organs (liver, kidneys, lungs, bone marrow).

In the vast majority of cases, the origin of RCMP is secondary, due to other reasons, among which the most common are:

1) amyloidosis - a disease associated with a violation of protein metabolism in the body; at the same time, in the tissues of various organs, an abnormal protein (amyloid) is formed and deposited in large quantities; when the heart is damaged, amyloid causes a decrease in its contractility and extensibility;

2) hemochromatosis - a violation of iron metabolism in the body, accompanied by an increased content of iron in the blood, its excess is deposited in many organs and tissues, including the myocardium, thereby causing a decrease in its extensibility;

3) sarcoidosis - a disease of unknown etiology, characterized by the formation of cell clusters (granulomas) in organs and tissues; the lungs, liver, lymph nodes and spleen are most often affected; and developing granulomas in the myocardium lead to a decrease in its extensibility;

4) endocardial diseases (endocardial fibrosis, endocardial fibroelastosis, etc.), when there is a significant thickening and compaction of the endocardium, which also leads to a sharp limitation of myocardial extensibility. Fibroelastosis of the endocardium, in particular, can only occur in infants; this disease is not compatible with life due to the early development of severe heart failure.

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