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Hospital therapy. Classification of cardiomyopathies. Etiology of hypertrophic cardiomyopathy (HCM) (most important)

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11. Classification of cardiomyopathies. Etiology of hypertrophic cardiomyopathy (HCM)

Hypertrophic cardiomyopathy (HCM) is a non-coronary disease of the ventricular myocardium (mainly the left), characterized by massive hypertrophy of their walls with protrusion of the interventricular septum (IVS) into the cavity of the right ventricle, which can be significantly thickened, a decrease in the internal volume of the ventricles, normal or increased contractility of the ventricular myocardium and impaired relaxation (diastolic dysfunction).

The most common is isolated hypertrophy of the interventricular septum (isolated hypertrophic subaortic stenosis - IHSS) or the apical part of the ventricles.

Classification. Classification of HCM by localization of hypertrophy (ED Wigle et al., 1985 with additions).

I. LV hypertrophy.

1. Asymmetric hypertrophy, in which myocardial hypertrophy of individual walls or segments of the ventricles occurs (including IVS hypertrophy - 90% with or without left ventricular outflow tract obstruction, midventricular hypertrophy - 1%, apical left ventricular hypertrophy - 3%, free wall hypertrophy left ventricle and posterior part of the IVS - 1%).

2. Symmetrical (concentric) hypertrophy of the left ventricle, when myocardial hypertrophy extends to all walls of the ventricles, occurs in 5% of cases.

II. Hypertrophy of the pancreas. In the case when myocardial hypertrophy prevents the normal outflow of blood from the ventricles of the heart, they speak of an obstructive form of HCM. In other cases, HCM is non-obstructive.

Etiology. The disease can be either congenital or acquired. Congenital HCM is inherited in an autosomal dominant fashion. Within the same family, various forms and variants of HCM can be observed. Most often, asymmetric hypertrophy of the interventricular septum is inherited.

The acquired form of HCM occurs in elderly patients with a history of arterial hypertension. The prevalence is 0,02-0,05%. The reasons for the development of acquired HCM are not fully understood. According to one of the proposed hypotheses, individuals with acquired HCM in the prenatal period develop a defect in the adrenergic receptors of the heart involved in the regulation of cardiac activity, in particular heart rate. As a result, the sensitivity to norepinephrine and similar hormones, which increase the heart rate, is significantly increased, which affects the development of myocardial hypertrophy in them, and eventually HCM.

pathological picture. Disoriented, irregular, chaotic arrangement of cardiomyocytes and myofibrils in cardiomyocytes, myocardial fibrosis is a violation of the architectonics of the heart muscle.

<< Back: Treatment and prevention of dilated cardiomyopathy (DCM)

>> Forward: Clinical picture and diagnosis of hypertrophic cardiomyopathy (HCM)

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