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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.
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.
RCMP (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 accumulate 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).
The 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 predominant lesion of 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 large 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 murmur 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 an 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 central hemodynamic parameters, 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 region of the papillary muscles are determined. Signs of insufficiency of mitral or tricuspid valves are noted.
In Leffler's endomyocardial fibroelastosis, severe eosinophilia and bronchospastic syndrome are possible, which, in combination with
Black heart enlargement and heart failure symptoms help to make a correct diagnosis.
Diagnostics. Recognizing the RCMP is extremely difficult. It is possible to speak confidently with 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, 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.
- 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
- 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. Hyperosinophilic
- 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,
- 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
- 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.
- 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
- 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
- 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
- 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
- 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
- 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, i.e. interstitial tissue of the alveolar walls, consisting of the basement membrane of the endothelium and epithelial cells, collagen fibers, elastic structures, proteoglycans, fibroblasts,
IBC Code: 142.0.0 Dilatation
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.
- 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
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
In recent years, cardiomyopathy has attracted increasing attention from 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
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