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Restrictive Cardiomyopathy



Restrictive CMP is associated with a decrease in tensile properties of the ventricular walls with manifestations of signs of hypodiastolia and symptoms of congestion in the major and minor circulation.

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

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

1. Hypereosinophilic parietal fibroplastic endocarditis Leflera.

2. Endomyocardial fibrosis (Davis disease).

Stage.

I. Necrotic.

Ii. Thrombotic.

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

Ultrasonography reveals dilatation of the cardiac cavities, a decrease in myocardial contractility. On the radiograph determine the increased size of the heart or its departments.

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

Cardiomyopathy with mitochondrial pathology. These include the following:

1) Cairns-Sayre syndrome;

2) MELAS syndrome;

3) MERRF syndrome;

4) Bart's syndrome;

5) carnitine CMP;

6) histiocytic CMP;

7) CMP with a deficiency of the P-complex chain of respiratory enzymes.

Criteria for CMP with mitochondrial pathology.

1. Extracardiac:

1) infantile somatotype (3-5 centile);

2) muscle weakness;

3) decrease in tolerance to an exercise stress;

4) visual impairment (ptosis), hearing;

5) stroke-like episodes;

6) periodic neutropenia;

7) persistent enlargement of the liver;

8) high levels of lactate and pyruvate;

9) increased excretion of organic acids;

10) a decrease in the content of blood carnitine - acidosis.

2. Cardiac:

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

2) the combination of hcmp, dcmp, fibroelastosis;

3) detection of hcmp at an early age;

4) the family nature of the disease;

5) giant T waves on the ECG in the left pectoral leads.

Cairns-Sayre syndrome.
Debut to 20-30 years. Symptoms: CMP with the development of a complete atrioventricular block, the formation of GKPM and DKMP, ophthalmoplegia with ptosis, retinopathy, delay of physical and sexual development, valgus deviation of the foot, cerebellar ataxia.

MELAS syndrome (mitochondrial myopathy-encephalopathy-lactate acidosis, stroke-like episodes). Debut between 6 and 10 years. Symptoms: convulsions, headache, vomiting, anorexia, dementia.

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

Bart's syndrome. Debut in 5-7 months of life. Symptoms: weight and height of 3-5 centile, growth retardation; lag of bone age at 1-2 years; skeletal myopathy; neutropenia; GKMP, DCM.

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 T teeth (above R) in the left chest leads.

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

CMP with deficiency of the P-complex chain of respiratory enzymes. Debut - after 9 years. Symptoms: ophthalmoplegia, encephalomyopathy, ataxia, myoclonic jerking, secondary carnitine deficiency, lactate acidosis, HCM, DCM, AKMP.

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Restrictive Cardiomyopathy

  1. RESTRICTIVE CARDIOMYOPATHY
    Definition Restrictive cardiomyopathy is an infiltrative or fibrous myocardial lesion, which is characterized by rigid, uncompliant ventricular walls, a decrease in the 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.
  2. Restrictive cardiomyopathy.
    This is a primary or secondary myocardial disease, characterized by diastolic dysfunction, lack of dilatation or ventricular hypertrophy, the presence of increased systemic and pulmonary venous pressure. Restrictive cardiomyopathy occupies 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 WHO experts, two diseases are attributed to restrictive cardiomyopathy: endomyocardial fibrosis and Löffler endo-carditis. There is a term that combines 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 (RCMP) is a rare myocardial disease with frequent involvement of the endocardium, which is characterized by impaired filling of one or both ventricles with a decrease in their diastolic volume with an unchanged wall thickness. Due to the deterioration of the diastolic properties of the ventricle, after a short period of rapid filling, further blood flow into it is almost
  5. 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 in two diseases differs little and is characterized by a sharp thickening of the endocardium in combination with ventricular myocardial hypertrophy, the cavities of which
  6. Primary idiopathic restrictive cardiomyopathy
    A rare form of non-infiltrative myocardial disease with restrictive-type diastolic dysfunction. The absence of LVH and systolic dysfunction is characteristic. Often inherited and associated with skeletal myopathy. The disease usually occurs sporadically, but can be inherited in an autosomal dominant manner. The appearance of a rigid ventricle may be due to pathology.
  7. Cardiomyopathy. Hypertrophic cardiomyopathy (code 142.0)
    The term "cardiomyopathy" refers to a state of unknown etiology, the most important signs of which are cardiomegaly and heart failure; this name excludes heart disease resulting from valve damage, impaired coronary blood flow, and hypertension in the large and small circulation. Etiology. Clinical and anatomical forms. Prospective
  8. Restrictive lung diseases
    Causes of restrictive lung disease Causes of acute restrictive disease: • ????? pulmonary edema; • ???? ARDS; •????aspiration; • ???? neurogenic edema; • ????? opioid overdose; • ???? congestive myocardial insufficiency; • ???? pleural effusion; •????pneumothorax; • ???? increase mediastinum; • ???? pneumomediastinum. Chronic lung disease leading to restrictive
  9. Restrictive lung diseases
    Restrictive diseases are characterized by a decrease in lung compliance. Lung volumes are below normal, while the volumetric flow rate on the exhalation is not reduced. Thus, FEV1 and FZHEL are reduced, but the value of the OFVch / FZHEL ratio 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 diseases
    There are two types of diffuse lung lesions. These are obstructive processes, affecting mainly the airways and characterized by an increase in resistance to the passage of air due to partial or complete obstruction at any level (from the trachea to the respiratory bronchioles), and restrictive processes that are associated with a decreased expansion of the lung parenchyma during inhalation and
  11. Restrictive type ODN
    This type of ARF is characterized by a decrease in the surface of the alveolar membrane due to the restrictive or restrictive effect on the lung parenchyma of various factors. They can be due both to changes in the lung parenchyma itself and to 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 team of non-communicable diseases is considered, characterized by predominantly diffuse and usually chronic changes that mainly affect the stroma of the lungs, interstitial tissue of the alveolar walls, consisting of the basement membrane of the endothelium and epithelial cells, collagen fibers, elastic structures, proteoglycans, fibroblasts,
  13. Cardiomyopathy
    IBC Code: 142.0.0 Dilatation
  14. Cardiomyopathy
    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 cardiomyopathy was given: "Cardiomyopathy is an acute, subacute or chronic lesion of the heart muscle of unknown or unclear etiology, often combined with a lesion of the endocardium, and sometimes the pericardium." This definition is accepted.
  15. CARDIOMYOPATHY
    - myocardial damage of unknown or unclear etiology, in which the dominant signs are cardiomegaly and heart failure, excluding the processes of damage to the valves, systemic and pulmonary vessels. Clinical classification 1. Congestive (congestive) cardiomyopathy, or primary myocardial disease. 2. Hypertrophic cardiomyopathy: a) without obstruction
  16. Cardiomyopathy.
    Cardiomyopathies are diseases of the myocardium 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 cardiomyopathy; specific cardiomyopathy. Dilatation
  17. CARDIOMYOPATHY.
    In recent years, cardiomyopathy has attracted increasing attention of cardiologists due to the fact that they have become more frequently diagnosed and, apparently, their true frequency has increased significantly. According to WHO, the name "cardiomyopathy" refers to a narrow group of myocardial lesions of unknown etiology, the most important manifestations of which are cardiomegaly and progressive cardiac
  18. CARDIOMYOPATHY
    Another group of non-coronary 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 be considered as independent primary forms of heart damage of unclear or controversial etiology with progressive adverse
  19. E.N. Amosov. Cardiomyopathy 1999
    Terminology and classification of cardiomyopathy, their place among other diseases of the myocardium. Dilated cardiomyopathy. Hypertrophic cardiomyopathy. Fibroplastic parietal
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