Cardiomyopathy

Cardiomyopathy is a complex of non-inflammatory cardiological diseases in which the heart muscle is affected. The term "cardiomyopathy" comes from three Greek words - kardia, which means the heart, myos - muscle and pathos - pathology, disease. The etiology of this symptom complex is still unclear, but it has been established that the disease is not provoked by hypertension, ischemic heart disease, and has no relation to heart defects. All the changes that are observed in the CML, can cause quite serious pathological disturbances in the work of the myocardium, which is responsible for the normal rhythm of the heart. The rhythm of contractions is based on the features of the structure of the myocardium muscular tissue, dystrophic, sclerotic lesions of it can lead to heart failure, arrhythmia and other cardiopathologies.

[1], [2], [3], [4], [5], [6], [7]

Cardiomyopathy: history of the disease and classification

Terminologically, cardiomyopathy was formed not so long ago. In the middle of the last century, in the 60s, cardiologist Bridgen conducted a study of heart disease of unclear etiology. All cardiac pathologies that were studied were characterized by an increase in the heart muscle, a decrease in circulatory activity, and death. Clinical cases were not related to IHD, rheumatism, hypertension or cardiopores. Thus, Bridgen was the first to suggest the similar pathologies of the myocardium by cardiomyopathies. Only in 1995, the disease "cardiomyopathy" was clearly defined, and a standard classification of the ILC was developed. Specific cardiomyopathy of the stage is called all myocardial dysfunctions of a specific etiology (ischemic cardiomyopathy, metabolic, hypertensive and other forms). If the etiology of the disease is not clear, then cardiomyopathy belongs to the primary category. In addition, WHO (World Health Organization) proposed uniform forms of the disease, corresponding to the pathophysiology and pathological anatomy of the development of the disease: 

• Dilatation - the cavity of the left ventricle of the heart is enlarged, the ability to contract muscle is reduced significantly (systole reduction). It is most common. 
• Hypertrophic - significantly thicken, the walls of the left ventricle grow, respectively, the function of relaxation (diastolic) decreases. 
• Arrhythmogenic (pancreatic) - a change in the structure of the right ventricle (less often the left), fibro-fatty degeneration of myocardial tissue. It is rarely found or diagnosed as a subspecies. 
• Restrictive - the elasticity of the walls of the myocardium decreases because of fibrous degeneration, infiltrates, respectively, the function of relaxation decreases. This form is less common than others, but there is evidence that RCMW is not diagnosed precisely because of the similarity of symptoms with other diseases or because of poor knowledge. 
• Cardiomyopathy is uncategorized - pathologies that, by specificity, can not be identified in any particular group. This option is practically not studied.

[8], [9], [10], [11], [12], [13], [14], [15], [16]

Cardiomyopathy: a description of the forms of the disease

Dilated cardiomyopathy is the most common type of disease, which occupies more than 60% of all diagnosed cases of cardiomyopathies. Dilation (from Latin dilato - to expand) leads to abnormal myocardium contraction dysfunction due to the expansion of the cavities (chambers). Cardiomyopathy dilated form is often called "stagnant." As a rule, DCM is diagnosed as a pathology of the left cardiac ventricle, less often the right ones are affected. The walls of the tissues remain unchanged, thickening or fibrous degeneration is not observed. Most often, dilated cardiomyopathy is a genetically determined disease, and infectious, autoimmune diseases, intoxication, including alcoholic, dystrophic muscle pathologies, are also called among the factors provoking the dilated form of cardiomyopathy.

DCM is diagnosed with a comprehensive examination, which necessarily includes an electrocardiogram, a heart ultrasound, an X-ray. Also, to clarify the diagnosis is important anamnestic information and laboratory tests of blood serum. The main diagnostic method that confirms the dilated form of the disease is ultrasound, all other diagnostic measures are not considered specific, rather they help to build a therapeutic strategy.

The clinical manifestations of dilated cardiomyopathy are very similar to those of heart failure. Typical signs are shortness of breath with a small load, swelling, a decrease in overall activity, pallor and cyanosis (acrocyanosis) of the skin, especially characteristic of cyanosis for the skin of the fingertips. Arrhythmia, atrial fibrillation, progressive cardialgia can lead to thromboembolism and even death. The prognosis of this disease is unsuccessful, since even with proper medication the mortality is about 40%, if cardiomyopathy of the dilated form is diagnosed late, then the mortality rate is 65-70%. DKMP and pregnancy are incompatible, since the percentage of deaths in these cases is almost 90%.

Therapeutic measures primarily solve the compensatory problem: it is necessary to ensure the rhythm of the heartbeats and minimize the manifestations of heart failure. It is also important to prevent possible serious complications. The appointment of ACE inhibitors (angiotensin-converting enzyme) - enalapril, enap, captopril, berlipril is shown. Also cardioselective beta-blockers - timolol, metoprolol, diuretics and anticoagulants - angioks, verpharin, and fractiparin are also effective. Acute conditions of the disease are medically supervised in hospital settings, in cases of exacerbation, surgical intervention is possible up to organ transplantation. 

Hypertrophic form of cardiomyopathy is characterized by abnormal enlargement (thickening) of the walls of the left cardiac ventricle without appreciable dilation of the cavity. Thus, the normal diastolic activity of the muscle is disrupted, the ventricle becomes denser, it does not stretch well, provoking increased intracardiac pressure. Among the causes of HCMC is also called family predisposition, which manifests itself in pathological gene defects that do not cope with the synthesis of protein compounds of the myocardium. Cardiomyopathy of the hypertrophied form can be congenital, however it is most often diagnosed in the pubertal period, when the symptomatology becomes obvious.

Cardiomyopathy hypertrophied form is quickly identified and differentiated from other forms. Diagnosis is carried out on the basis of the presented symptoms, the collection of anamnestic information (including family information). Stethoscopic listening clearly determines systolic noises. The main method that specifies the form of cardiomyopathy is ultrasound, ECG information is also important, where changes in rhythm and conductivity are visible. The X-ray, as a rule, shows increased intracardiac pressure in the left branch of the pulmonary trunk (artery).

Clinical manifestations characterized by hypertrophied cardiomyopathy are rapidly progressing symptoms of heart failure. Cardialgia, frequent dizziness until fainting, swelling and shortness of breath. Extrasystolic disorders, tachycardia (paroxysms), endocarditis and thromboembolism often develop. These signs appear, unfortunately, already at the stage of the developed disease, whereas the onset of myocardial infarction is most often asymptomatic. This explains the sudden deaths in fairly young people who do not have visible cardiological pathologies, especially sudden deaths in people who exercise in case of severe physical overload.

Therapeutic actions with diagnosed HCM are aimed at compensating the work of the left heart, eliminating the inadequacy of functioning. Cardiomyopathy of the hypertrophied form is supervised by the use of active beta-blockers, such as verapamil, isoptin, and phinoptin. Cardiotonics - glycosides, such as strophanthin, korglikon, rauwolfia alkaloids as antiarrhythmics - rhyomodan, rhythmelin, diuretics are also effective. In the acute form of the disease is shown pacing, surgical intervention. 

Restrictive variant of cardiomyopathy. This type of myocardial infarction is rarely diagnosed, possibly because of the low knowledge of the disease and because of the asymptomatic course of the disease. With this form of the disease, the relaxation of the myocardium is broken, the contraction decreases, the muscle becomes rigid due to the fibrous degeneration of the inner wall (endocardium). This leads to insufficient filling of the left side of the heart (the ventricle) with blood flow. The tissue of the walls of the ventricle does not change structurally, it is not thickened, the expansion of the cavity is not revealed. If HCMs are accompanied by serious pathologies, such as lymphoma, hemochromatosis (pathological accumulation of iron in tissues), the symptoms become more pronounced, and the development of the disease is rapid.

Restrictive form of cardiomyopathy is diagnosed similarly to other forms of CML. The main demonstration method is ultrasound scanning of the heart, as well as angiocardiography. The radiograph shows a significant increase in the size of the atria, although the heart itself does not change either shape or size. An electrocardiogram with this form of cardiomyopathy is not informative, laboratory tests of blood serum are additional and necessary for the choice of drugs, but not indicative in a diagnostic sense.

Restrictive form of cardiomyopathy most often occurs without pronounced signs. Symptomatics is manifested in the last stage of the disease, or when combined with other pathologies. Among the obvious symptoms are the following: 

• Dyspnea with little physical exertion; 
• Frequent sore throats; 
• Nocturnal paroxysmal attacks of dyspnea; 
• Edema; 
• Ascites.

Cardiomyopathy of this form is difficult to treat, basically the therapeutic strategy is aimed at eliminating acute pathological symptoms, the next stage of treatment is extremely unpredictable. Cardiomyopathy of the restrictive form proceeds asymptomatically, and the treatment begins at the last terminal stage, when the medicamental help is practically powerless. Basic therapy solves the following problems: 

• Minimization of pulmonary overload; 
• Decreased intracardiac pressure; 
• Activation of systolic function (pumping); 
• Minimizing the risk of blockage of blood flow - thromboembolism.

The prognosis for this form of ILC is very unfavorable, statistics show that about 50% of patients die within two to three years after diagnosis. Cardiomyopathy of the restrictive form is rapidly progressing, diastolic dysfunction develops rapidly. The appointment of cardioglycosides, vasodilating drugs (vasodilators), diuretics is shown. Timely drug therapy can prolong the life of patients for several years, also facilitates the state of endocardectomy or prosthetic valve. However, surgical intervention is associated with an objective risk of increased lethality.

Cardiomyopathy is dangerous in all forms and stages, therefore an early diagnosis plays an important role in the effectiveness of treatment and its outcome. In cases where heart failure can not be controlled with the help of therapeutic measures, heart transplantation is performed.

[17], [18], [19], [20], [21]