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Restrictive cardiomyopathy



Restrictive CMP is associated with a decrease in the extensibility of the walls of the ventricles with the manifestation of hypodiastolic symptoms and symptoms of stagnation in the large and small circle of circulation.

Primary myocardial (isolated myocardial damage, similar to that of DCM).

Endomyocardial (thickening of the endocardium and infiltrative necrotic and infiltrative changes in the myocardium).

1. Hypereosinophilic parietal fibroplastic endocarditis of Lefler.

2. Endomyocardial fibrosis (Davis disease).

Stages.

I. Necrotic.

II. Thrombotic.

III. Fibrotic: on ECG - decrease in voltage of teeth, violation of the processes of conduction and excitation, change in the final part of the ventricular complex.

When ultrasound is found dilatation of the heart cavities, a decrease in the contractility of the myocardium. On the roentgenogram determine the increased size of the heart or its parts.

Arrhythmogenic CMS of the right ventricle. Clinic: ventricular extrasystoles, paroxysmal tachycardia.

Cardiomyopathy in mitochondrial pathology. These include the following:

1) Cairns-Seir syndrome;

2) MELAS syndrome;

3) MERRF syndrome;

4) Barth's syndrome;

5) carnitine CMP;

6) histiocytic cMYP;

7) ILC with deficiency of the P-complex of the respiratory enzyme chain.

Criteria for CML in mitochondrial pathology.

1. Extracardiac:

1) infantile somatotype (3-5 centiles);

2) muscle weakness;

3) decreased tolerance to physical activity;

4) visual impairment (ptosis), hearing;

5) stroke-like episodes;

6) periodic neutropenia;

7) persistent enlargement of the liver;

8) high level of lactate and pyruvate;

9) increased excretion of organic acids;

10) decrease in the blood carnitine content - acidosis.

2. Cardiac:

1) disorders of the conduction system of the heart malignant, ventricular arrhythmias are characteristic of young children;

2) a combination of HCM, DCMP, fibroelastosis;

3) detection of HCM at early age;

4) the family nature of the disease;

5) giant T-wave on the ECG in the left thoracic leads.

Cairns-Seyr syndrome.
The debut is for 20-30 years. Symptoms: CMS with the development of full atrioventricular block, possibly formation of GKPM and DCMP, ophthalmoplegia with ptosis, retinopathy, delay in physical and sexual development, valgus deviation of the foot, cerebellar ataxia.

MELAS syndrome (mitochondrial myopathy-encephalopathy-lactate acidosis, stroke-like episodes). The debut is between 6 and 10 years old. Symptoms: cramps, headache, vomiting, anorexia, dementia.

MERRF syndrome (myoclonus-epilepsy and cerebral infarction, RRF-fibers). The debut is from 3 to 63 years. Symptoms: myoclonus-epilepsy, ataxia, dementia (due to multiple cerebral infarctions), hearing loss, muscle weakness, HCM.

Barth's syndrome. Debut in 5-7 months of life. Symptoms: weight and height of 3-5 centiles, growth retardation; lag of bone age for 1-2 years; skeletal myopathy; neutropenia; HCMC, DCMP.

Carnitine CMP. Debut in 3-5 months. Often sudden death due to metabolic stress. Symptoms: myocardial hypertrophy with dilatation of the left ventricular cavity, endocardial fibroelastosis, ECG giant teeth T (above R) in the left thoracic leads.

Histiocytic CMP (cytochrome B deficiency). The debut - in 3 weeks - 1 year. More often in girls. Despite the treatment, they die. Symptoms: tachyarrhythmias, ventricular fibrillation, myocardial hypertrophy with dilatation of the left ventricular cavity, fibroelastosis of the heart.

ILC with deficiency of the P-complex of the respiratory enzyme chain. Debut - after 9 years. Symptoms: ophthalmoplegia, encephalomyopathy, ataxia, myoclonic twitchings, secondary carnitine deficiency, lactate acidosis, HCM, DCMP, ACMP.

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Restrictive cardiomyopathy

  1. RESTRICTIVE CARDIOMYOPATHY
    Definition Restrictive cardiomyopathy is an infiltrative or fibrotic lesion of the myocardium that is characterized by rigid, obstructive ventricular walls, a decrease in filling and diastolic volume of one or both ventricles with normal or almost unchanged systolic function and wall thickness. At the heart of the disease is widespread interstitial fibrosis.
  2. Restrictive cardiomyopathy.
    This is a primary or secondary disease of the myocardium, characterized by diastolic dysfunction, the absence of dilatation or hypertrophy of the ventricle, the presence of increased systemic and pulmonary venous pressure. Restrictive cardiomyopathy holds a special place among all myocardial diseases and is rare. ETIOLOGY. The cause of restrictive cardiomyopathy is considered fibroplastic
  3. RESTRICTIVE CARDIOMYOPATHY.
    According to the conclusion of WHO experts, two diseases are classified as restrictive cardiomyopathy: endomyocardial fibrosis and endo-carditis of Löffler. There is a term that unites both diseases - "Endomyocardial disease". Diseases are found in countries with hot climates. Endemic foci have been identified in Uganda, Tanzania, Zambia, Mozambique,
  4. Restrictive cardiomyopathy
    Restrictive cardiomyopathy (RSCM) is a rare disease of the myocardium with frequent involvement of endocardium, which is characterized by a violation of filling one or both ventricles with a decrease in their diastolic volume with unchanged wall thickness. Due to the deterioration of the diastolic properties of the ventricle, after a short period of rapid filling, the further flow of blood into it is practically
  5. Restrictive cardiomyopathies
    Restrictive cardiomyopathy (RCMW) is known in two versions of Fanee considered as two independent pathological processes) - endocardial fibrosis and endometriocardial fibroelastosis * efler. The pathomorphological pattern of the two diseases is not very different and is characterized by a sharp thickening of the endocardium in combination with myocardial hypertrophy of the ventricles whose cavities
  6. Primary idiopathic restrictive cardiomyopathy
    A rare form of non-infiltrative myocardial disease with restrictive diastolic dysfunction. Characteristic is the absence of LVH and systolic dysfunction. It is often inherited and associated with skeletal myopathy. Usually, the disease arises sporadically, but can be inherited by an autosomal dominant type. The occurrence of a rigid ventricle can be a consequence of pathology
  7. Cardiomyopathy. Hypertrophic cardiomyopathy (cipher 142.0)
    The term "cardiomyopathy" refers to the state of unknown etiology, the most important signs of which are cardiomegaly and heart failure; From this name, heart diseases that result from damage to the valves, coronary artery disease, hypertension of the large and small circle of circulation are excluded. Etiology. Clinical and anatomical forms. Proposed
  8. Restrictive pulmonary disease
    Causes of restrictive lung pathology Causes of acute restrictive pathology: • edema of the lungs; • ???? ARDS; •????aspiration; • ???? neurogenic edema; • Overdose of opioids; • ???? congestive myocardial insufficiency; • ???? pleural effusion; •????pneumothorax; • Increase of the mediastinum; • ???? pneumomediastinum. Chronic lung diseases, leading to restrictive
  9. Restrictive pulmonary disease
    Restrictive diseases are characterized by a decrease in the extensibility of the lungs. Pulmonary volumes are below normal, while the volumetric flow rate during exhalation is not reduced. Thus, FEV1 and FVC are reduced, but the value of the ratio OFFh / FVC remains normal. Restrictive diseases include many acute and chronic pathological conditions of the lungs, as well as lesions of the pleura, chest wall, diaphragm and
  10. Obstructive and restrictive lung disease
    There are two types of diffuse lesions of the lungs. These are obstructive processes that mainly affect the airways and are characterized by increased resistance to air flow due to partial or complete obstruction at any level (from the trachea to the respiratory bronchioles) and restrictive processes, which are characterized by a reduced expansion of the lung parenchyma during inspiration and,
  11. Restrictive type ODN
    This type of ODN is characterized by a decrease in the surface of the alveolar membrane due to restrictive or restrictive effects on the lung parenchyma of various factors. They can be caused by both changes in the pulmonary parenchyma itself and extrapulmonary causes: lung atelectasis, hydrothorax, pneumothorax, diaphragmatic hernia, systemic lupus erythematosus, fibrosing
  12. Diffuse interstitial (infiltrative and restrictive) lung diseases
    In this section of the chapter, a collection of noncommunicable diseases, characterized mainly by diffuse and usually chronic changes, which mainly affects the stroma of the lungs, is considered. interstitial tissue of the alveolar walls consisting of the endothelial basement membrane and epithelial cells, collagen fibers, elastic structures, proteoglycans, fibroblasts,
  13. Cardiomyopathies
    142 142.0 Dilated cardiomyopathy 142.1 Obstructive hypertrophic cardiomyopathy (hypertrophic subaortic stenosis) 142.2 Other hypertrophic cardiomyopathy 142.3 Endomyocardial (eosinophilic) disease (endomyocardial tropical fibrosis, endocarditis of Leffler) 142.4 Endocardial fibroelastosis (congenital cardiomyopathy) 142.5 Other
  14. Cardiomyopathies
    The term "cardiomyopathy" was first used in 1957 to refer to a group of myocardial diseases of unknown etiology. In 1972, the following definition of cardiomyopathies was given: "Cardiomyopathy is an acute, subacute or chronic lesion of the cardiac muscle of unknown or obscure etiology, often combined with endocardial damage, and sometimes pericardium." This definition is accepted
  15. CARDIOMYOPATHY
    - myocardial damage of unknown or unclear etiology, in which cardiomegaly and heart failure are the dominant signs, excluding the processes of damage to valves, systemic and pulmonary vessels. Clinical classification 1. Congestive (congestive) cardiomyopathy, or primary myocardial disease. 2. Hypertrophic cardiomyopathy: a) without obstruction of the pathways
  16. Cardiomyopathy.
    Cardiomyopathies are myocardial diseases accompanied by cardiac dysfunction. In accordance with the classification proposed by the WHO Expert Committee (1995), the following are distinguished: dilated cardiomyopathy; hypertrophic cardiomyopathy; restrictive cardiomyopathy; arrhythmogenic right ventricular cardiomyopathy; unclassified cardiomyopathies; specific cardiomyopathies. Dilatational
  17. CARDIOMYOPATHY.
    In recent years, cardiomyopathies have attracted increasing attention of cardiologists due to the fact that they became more often diagnosed and, apparently, their true frequency increased significantly. According to the conclusion of WHO, the name "cardiomyopathy" refers to a narrow group of myocardial lesions of unknown etiology, the most important manifestations of which are cardiomegaly and progressing cardiac
  18. CARDIOMYOPATHY
    Another group of noncoronogenic myocardial lesions, the most severe of all, both in terms of diagnosis and clinical manifestations, and in terms of treatment, is cardiomyopathy. At the suggestion of W.Brigden / 1957 / and J. Goodwin (1961), the term "cardiomyopathy" should consider independent primary forms of heart involvement of an unclear or controversial etiology with progressive adverse
  19. E.N. Amosov. Cardiomyopathy, 1999
    Terminology and classification of cardiomyopathies, their place among other myocardial diseases. Dilated cardiomyopathy. Hypertrophic cardiomyopathy. Fibroplastic parietal
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