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Odessa National Medical UniversityDepartment of Internal medicine №1 with the course of cardiovascular diseaseMETHODICAL RECOMMENDATIONSof the practical lesson for students Discipline "Internal medicine, including endocrinology"Topic "Cardiomyopathies"Course IV Faculty: Medical, International Specialty: 222 " Medicine " Approved at the methodical meeting of the department ___________. Protocol № ______ Head of Department Prof. Y.I. Karpenko Odesa ?. Relevance of the topicHypertrophic cardiomyopathy (HCM) it is a primary myocardial damage characterized by severe myocardial hypertrophy Left ventricular (RV less), normal or reduced the size of the left ventricle cavity, significant impairment diastolic function ventricle and the frequent occurrence of cardiac arrhythmias. Dilated cardiomyopathy (DCM)- a primary lesion of the myocardium, characterized by marked dilatation of the cavities and impaired ventricular systole function. DCM term applies only to cases in heart disease, in which a significant dilatation of the cavities is not due to violation of the coronary circulation (coronary artery disease), congenital abnormalities, valvular heart disease, systemic and pulmonary hypertension, and pericardial disease.Restrictive cardiomyopathy (RCM) is a heterogeneous group of primary (idiopathic) or secondary heart diseases involving lesions endocardial and / or the myocardium, which leads to a pronounced fixed restriction (restriction) filling of the ventricles during diastole and development of diastolic dysfunction of one or both ventricular diastole and progressing CHF. II. Purpose of the activityTo be able to diagnose and perform therapeutic and preventive measures incardiomyopathy.To implement the goals are necessary basic knowledge:Know the anatomy and physiology of the cardiovascular system;Know technique anamnesis in patients with cardiovascular disease;Know techniques for objective examination of these patients;4. Know the basic methods of instrumental and laboratory research in cardiology and regulations; 5. To be able to carry out differential. diagnosis6. Know the methodology of diagnosis; 7. Know the basic principles of antimicrobial, antiviral, metabolc therapy and indications for surgical intervention.III. Tasks for self-study students to engageInformation to improve the level of basic knowledge can be found in the following books:Sources:Davidson’s “Principles of Practice of Medicine” 20th edition 2006, Elsevier limited.2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyo-pathy. The Task Force for the Diagnosis and Management of Hypertrophic Cardi-omyopathy of the European Society of Cardiology (ESC) // European Heart Jour-nal (2014) 35, 2733-2779Clinical protocol of care for patients with hypertrophic cardomyopathy; MOH Order from 03.07.2006 № 436IV. Control questions:The definition of "CMP" concept.The classification of the CMPEvaluation of patients with suspected CMP4. Diagnostic criteria of the CMP6. Differential diagnosis.7. Prevention and clinical examination. 8. The standard of care for patients with CMPV. Indicative map for selfitem numberThe content and sequence of learning activitiesNotes to the educational actions1. Know the diagnostic capabilities to detect CMPBe patient survey plan2.Know the basic clinical and instrumental and laboratory data of the CMPWrite CMP diagnosis criteria. The decision of test problems of the final control3.Be able to apply assimilated in the clinical situationWrite the clinical diagnosis of patients examined. Write recipes.Tasks for ISW. Student participation in the selection and production of illustrative material.Write a paper on the topic "Modern methods of treatment of the cardiomyopathy."Content of the topicHypertrophic cardiomyopathy (HCM) it is a primary myocardial damage characterized by severe myocardial hypertrophyof left ventricular (RV less), normal or reduced the size of the left ventricle cavity, significant impairment diastolic function ventricle and the frequent occurrence of cardiac arrhythmias. Distinguish asymmetrical and symmetrical forms of HCM. More common asymmetrical shape with predominant hypertrophy top, middle or bottom third of the IVS, the thickness of which may be 1.5-3 times the thickness of the rear wall and left ventricle exceeds15 mm. Sometimes IBE thickness of 50-60 mm. In some cases IVS hypertrophy combined with an increase in muscle mass of anterior oranterio-lateral ventricular wall, while the thickness of the rear wall remains nearly normal. Finally, in some cases, the predominant hypertrophy of the top (apical form of HCM) with a possible transition to the lower part of the IVS (Fig. 10.6, etc.) or the front wall of the left ventricle.For symmetrical HCM thickening characteristic almost identical front , rear walls of the left ventricleandIVS (concentricLV hypertrophy). Furthermore, in some cases, together with the described changes in the LV mayhypertrophied myocardium RV. Cardiac mass increases rapidly, reaching in some cases, 800-1000 gr. The cavity of the left ventricle is usually narrowed. Of particular interest are the cases of so-called obstructive HCM shape with asymmetrical (or total) lesion of IVS and LV outflow tract obstruction. In these cases, we talk about the presence of patients with idiopathic subaortic subvalvular (muscle) stenosis, which causes the most pronounced changes in intracardiac hemodynamics.Histological study of patients with HCM myocardium find some specific signs of this disease: - disoriented chaotic arrangement of cardiomyocytes; - fibrosis of the myocardium in the form of diffuse or focal development of the connective tissue in the heart muscle, and in many cases with the formation of extensive transmural scar and even fields; - a thickening of the walls of small coronary arteries due to hypertrophy of the smooth muscle cells and increase of fibrous tissue in the wall. HCM incidence is 2-5 per 100 000 population or 2-3 cases per 1000 young people (20-35 years). Forms predominate obstructive HCM, the detection rate of which is about 2-3 times greater than obstructive. Men get sick more often than women. The first clinical manifestation of the disease, usually occur in young age (20-35 years).EtiologyCurrently, you can only conditionally speak of HCM as a disease of unknown etiology. With today genetics found that at the origin of HCM are genetic factors, namely, abnormalities that are inherited or spontaneous mutations that function of contractile myocardial protein (b-myosin heavy chain, troponin T, troponin I, a-tropomyosin and protein C). The genes are located in chromosomes 1, 2, 7, 11, 14 and 15. A gene defect is changing the aminoacid sequence.In most cases, gene mutations known to lead to the synthesis of an abnormal heavy chain b-myosin, troponin T and less a-tropomyosin. Abnormal proteins lead to disruption of its structure and function. Thus, about half the cases of HCM is a family history, inheritance and gene abnormality occurs in an autosomal dominant manner. Approximately half of the close relatives of patients with familial hypertrophic cardiomyopathy (parents, siblings) areechocardiographicsigns of hypertrophy of the IVS. In other cases it is not possible to prove familial hypertrophic cardiomyopathy and diseases associated with spontaneous mutations in these genes may occur under the influence of this adverse environmental factors (HCM sporadic forms). Differentiation of familial and sporadic forms of HCM difficult. A certain value in the formation of the disease attached by action of neurohormonal factors: catecholamines, insulin, growth hormone, and thyroid dysfunction of the parathyroid glands.Pathogenesis1.diastolic dysfunction LV It is the basis of hemodynamic disorders in any form HCM (obstructive and non-obstructive). diastolic dysfunctiondue to the increase in rigidity and sclerotic hypertrophic cardiac muscle and a violation of the process of active ventricular diastolic relaxation. Natural Consequences LV diastolic filling are:- an increase in end-diastolic pressure in the left ventricle;- an increase in pressure in the LA and in the veins of the pulmonary circulation- LA extension (in the absence of LV dilation)- stagnation of blood in the pulmonary circulation (diastolic heart failure form)- reduction of cardiac output (in the later stages of the disease) resulting in difficulties diastolic ventricular filling, and decrease in EDV (decrease LV cavity).2. LV systolic function in patients with hypertrophic cardiomyopathy usually is not broken or even increased: the power reduction hypertrophied LVand the rate of expulsion of blood into the aorta usually increase sharply. EF Increases too. 3. Relative coronary insufficiency one of the characteristic features of HCM. Violations of the coronary blood flow caused by:- narrowing of the small coronary arteries due to hypertrophy of the smooth muscle cells and the development of the connective tissue in the arterial wall - increase in LV DAC, results in a pressure drop gradient between the aorta and the left ventricular cavity and, consequently, a decrease in coronary blood flow- - mismatch significantly increased muscle mass and left ventricular heart capillary channel;- an increase in myocardial oxygen demand with heart muscle hypercontraction.4. High risk of ventricular and supraventricular arrhythmias, and risk of sudden death due mainly expressed electrical inhomogeneity and instability of the ventricular and atrial myocardium that occur in patients with hypertrophic cardiomyopathy resulting mosaic arrangement focal myocardial portions, having different electrophysiological properties (hypertrophy, focal fibrosis, local ischemia). A certain value in the occurrence of arrhythmias has tensile dilated wall of LA and toxic effects of catecholamines in the myocardium of the ventricles.The magnitude of the pressure gradient and the degree of outflow tract obstruction significantly affect three main factors:1. Reduction of left ventricular myocardium.2. The amount of preload. 3. Size afterload. Therefore, all the factors that contribute to ventricular contraction, increase the outflow tract obstruction (physical activity, Tachycardia, psycho-emotional stress, the introduction of cardiac glycosides and other inotropic agents, gipercateholaminemia). In contrast, bradycardia or using drugs having a negative inotropic action (b-blockers, calcium channel blockers slow, disopyramide, etc.) may reduce the obstruction.Reduced preload leads to further reduce the volume of the ventricle and accordingly sizes and outflow tract obstruction accompanied by strengthening it. Therefore, the degree of obstruction increases with patient sudden transition from a horizontal to a vertical position, with Valsalva maneuver, taking nitrates, tachycardia. On the contrary, the horizontal position of the patient, any increase in BCV reduce the degree of obstruction.Finally, reduction of afterload (for example, decrease in blood pressure when receiving arterial vasodilators) increases the left ventricular introducing tract obstruction, while its increase (rise in blood pressure, prolonged static loads, cooling action, administering mezatona, noradrenaline etc.) reduces intraventricular pressure gradient and the degree of obstruction. Remember1. In contrast to fixed obstruction at the valve aortic stenosis, hypertrophic cardiomyopathy with obstruction of the left ventricular introducing path is dynamic in nature.2. The degree of obstruction increases with:- increase LV contraction (physical load, activation of CAS, tachycardia, the use of inotropic drugs and sympathomimetics)- a decrease in preload (vertical position of the patient, Valsalva maneuver, hypovolemia, taking nitrates)- a decrease in afterload (decreased systemic vascular resistance and blood pressure). The dynamic nature of the outflow tract obstruction in HCM patients explains the fact that the value of intraventricular pressure gradient continuously varies at one and the same patient, in particular under the action of the above factors. It should be noted that the intraventricular obstruction can develop not only in the hypertrophy of the IVS, but with the defeat of the left ventricle of other departments. In some cases it is also possible obstruction introducing pancreatic tract.RememberIf HCM observed the following changes in intracardiac hemodynamics: 1. Severe LV hypertrophyand reducing its cavity size. 2.diastolic dysfunctionof LV, which leads to increased pressure in the LA cavity, the veins of the pulmonary circulation and pulmonary congestion. 3. Dilation of LA, due to increased left ventricular filling pressure and medium pressure LA. 4. Reducing the cardiac output at a relatively normal or increased contractility infarction and left ventricular systolic function. 5. Relative coronary insufficiency. 6. Dynamic introducing LV tract obstruction (obstructive HCM when form).CliniccomplaintsThe clinical manifestations of HCM determined mainly described in the previous section disorders of intracardiac hemodynamics. For a long time the disease can be asymptomatic, and objective signs of hypertrophic cardiomyopathy detected by chance. The first clinical manifestations often occur between the ages of 25-40 years.Dyspnea first appears on exertion, and then alone. In some cases, shortness of breath may be increased by moving the patient in an upright position, especially in patients with obstructive form of HCM, which is associated with a decrease in venous blood flow to the heart and an even greater reduction in LV filling.Dizziness and fainting explained transient disturbances of brain perfusion and caused a decrease in cardiac output and by the presence of obstruction introducing LV tract. Dizziness and fainting may patient during rapid transition from a horizontal to a vertical position, it reduces the amount of preload and also increases outflow tract obstruction. Syncope triggered as physical exercise, straining, and even food. In the latter case there is often a vasodilation, reduced afterload and an increase in the outflow tract obstruction.Angina pectoris in patients with hypertrophic cardiomyopathy result from narrowing of the coronary arteries and small changes in intracardiac hemodynamics described above. Often appears in patients with angina pectoris during the physical exertion or emotional stress. Interestingly, no nitrates reception relieves angina, but rather, may worsen the patient's condition, as the degree of obstruction increases and enhancesdiastolic dysfunction LV. Palpitations and irregular heart work may be associated with the occurrence of atrial fibrillation, supraventricular and ventricular arrhythmias and paroxysmal tachycardia. It should be emphasized that sometimes the first and last manifestation of HCM can be sudden cardiac death. physical examinationIn patients with obstructive form of HCM is objective evidence of disease may be absent for a long time until it develops a pronounced stagnation of blood in the pulmonary circulation. In patients with obstructive HCM objective signs of the disease manifest themselves early enough during the examination of the cardiovascular system.Auscultation of the heart. Auscultation of the heart reveals the most specific signs of obstructive form of hypertrophic cardiomyopathy. Basic heart sounds are not often changed, perhaps splitting tone I in connection with the non-synchronous contraction of the left and right ventricles.Accent II tone appears on the pulmonary artery with a significant increase in pulmonary artery pressure. Often at the top of the heart is listened presystolic gallop rhythmdue to the appearance of pathological IV tone. In some of the patients have a paradoxical splitting of the II tone of the aorta.Systolic noise is the main feature of auscultatory obstructive HCM. It reflects the occurrence of the dynamic pressure gradient between the left ventricle and the aorta. The noise is loud, coarse, usually auscultated along the left sternal border and not carried out at the neck vessels.Systolic noise, as well as itself outflow tract obstruction, increased during exercise, decrease in blood pressure, decrease venous blood flow to the heart (e.g., by the action of nitrate). The attenuation observed in systolic noise reducing myocardial contraction (reception b-blockers), increased blood pressure, as well as horizontal position of the patient. It should be borne in mind that in some patients with systolic murmur is determined only after physical exertion.At the top is often auscultated systolic murmur of mitral regurgitation. It is soft, gentle, starts immediately after I pitch is holosystolic character and is held in the armpit.Arterial pulse and blood pressure. In severe cases of obstructive HCM determined intercadence. Often in patients with HCM is a concomitant AH.DiagnosticselectrocardiographyAlmost all patients with HCM detect ECG changes, and in some cases long before echocardiographicsigns of hypertrophy of the left ventricle interventricular septum or other departments. The most important are the following changes:1. Signs left ventricular hypertrophy. 2. Nonspecific changes ventricular complex (depression RS-T segment and T wave inversion).3. Signs and the overload hypertrophy atrial (P-mitrale). 4. Pathological Q and complex QS. Another cause of pathological Q or complex QS are areas of fibrous tissue in the IVS, front or rear walls of the left ventricle. 5. supraventricular and ventricular arrhythmias. When ECG monitoring Holter changes detected in 80% of cases. Often recorded ventricular arrhythmias high grade, which are a precursor to ventricular fibrillation and sudden cardiac death. Sometimes there is a mismatch of ECG changes and echocardiographic data. Description HCM cases, long asymptomatic, only manifestation which are giant tine R S and deep at one or more chest leads. In other cases, the presence of severe hypertrophy (by echocardiography) was almost normal ECG.Echocardiographic study It is the main method to verify HCM. Most interesting is the ultrasound diagnosis of obstructive form of HCM with asymmetric hypertrophy of the IVS and the obstruction of the left ventricular outflow tract.Echo-symptoms of this form of hypertrophic cardiomyopathy are: 1. thickening of the interventricular septum and limiting its mobility. 2. Reducing the ventricular cavity and enlargement LA.3. systolic motion of the anterior leaflet of the mitral valve, resulting from a significant acceleration of blood flow in the narrowed portion of the LV outflow tract and suction venturi effect.4. systolic aortic cover flap mid systole and the appearance of the dynamic pressure gradient in the LV outflow tract.5. When Doppler examination revealed a high linear velocity of blood flow in the left ventricular outflow tract and the characteristic humped shape spectrogram transaortic blood flow velocity. 6. Signs of severe diastolic dysfunction LV. 7. hyperkinesia of posterior wallLV.8. Mitral regurgitation. These data can be obtained by two-dimensional echocardiography with upland or subcostal access, as well as the study of blood flow in the left ventricular outflow tract in the Doppler mode. radiography X-ray examination is not decisive for the diagnosis of HCM. Often heart contours are normal. In severe mitral regurgitation is determined by the expansion of the shadow of the LA. In severe pulmonary hypertension is a bulging of the second arc of the left cardiac contour (Conus pulmonalis), expansion of the lung root and radiological signs of venous (less frequently arterial) pulmonary hypertension.TreatmentHCM patients, especially obstructive form of the disease, it is recommended to avoid the considerable physical exertion, accompanied by tachycardia, an even greater deterioration of left ventricular filling in diastole and increasing intraventricular pressure gradient in the left ventricular introducing path. The basis of drug therapy in patients with HCM constitute b-blockers, which effect is manifested approximately 80% of patients. These drugs due to negative chronotropic action, reduce heart rate, diastolic and lengthen contribute some improvement in diastolic left ventricular filling. This reduces the pressure in the LA and veins of the pulmonary circulation and reduces congestion in the lungs.Patients with obstructive HCM form negative inotropic action b-blockers reduces the rate of ventricular contraction and thereby reduces the intraventricular pressure gradient. Clinical efficacy is expressed b-blockers in reducing dizziness, syncope, and dyspnea. In addition, b-blockers can prevent the occurrence of supraventricular arrhythmias and reduce the risk of ventricular arrhythmias, high grade, and sudden death.Dosages of these agents are selected strictly individually, trying to avoid an undesirable decrease in blood pressure, which can help increase the intraventricular pressure gradient. For example, the daily dose of propranolol ranging from 80 to 280 mg per day and higher.It should be noted that calcium channel blockers slow dihydropyridine contraindicated in patients with hypertrophic cardiomyopathy due pronounced antihypertensive and hypotensive action, and the ability to induce reflex tachycardia. Unfortunately, attempts to use for the treatment of HCM patients ACE inhibitors proved unsuccessful so far.When atrial fibrillation occurs, together with b-blockers useful to designate indirect anticoagulants (warfarin).Remember.When obstructive HCM contraindicated application form: - nitrate (reduced preload)- cardiac glycosides and other inotropic agents (increased blood ejection velocity in the aorta and prisasyvayuschi venturi effect)- calcium channel blockers slow dihydropyridine (reduced afterload). Surgical treatment is usually used in severe cases. When obstructive HCM with marked hypertrophy and IBE high pressure gradient operation is shown myotomy-mioektomii. It consists in the resection of a small portion of the proximal portion IBE. The operation usually eliminates symptoms outflow tract obstruction (syncope terminated), although other manifestations of hypertrophic cardiomyopathy are stored (angina, dyspnea and others.).Recently, to eliminate obstruction endure LV tract due to systolic closure of the anterior leaflet of the mitral valve and the IBE, spend mitral valve replacement. This operation is also effective and allows to eliminate obstruction introducing LV tract.Finally, for the treatment of patients with obstructive form of HCM is sometimes implanted permanent dual-chamber pacemaker. With the localization of intraventricular stimulating electrode in the apex changing the sequence of excitation and, consequently, reduction, namely: first, reducing the tip of the free wall of the left ventricle, and only in the last instance the basal parts of the IVS. This sequence of contraction of the heart allows you to reduce the signs of outflow tract obstruction.forecastForecast HCM serious enough. Sudden cardiac death occurs in 1-4% of patients per year, even higher incidence of sudden death in children (up to 6% per year).rememberThe main predictors of sudden death in HCM is considered - the young age of the patients, - cases of sudden death in the immediate family; - the presence of the patient paroxysms of ventricular tachycardia ECG, Holter monitoring detected. A small percentage of patients with HCM (approximately 10%) possible transformation of the disease in the dilatation CMP. In 10% of cases in patients with HCM develop a picture of infectiousendocarditis. Dilated cardiomyopathy (DCM)- a primary lesion of the myocardium, characterized by marked dilatation of the cavities and impaired ventricular systole function. The term DCM applies only to cases in heart disease, in which a significant dilatation of the cavities is not a consequence of a violation of the coronary circulation (coronary artery disease), congenital abnormalities, valvular heart disease, systemic and pulmonary hypertension, and pericardial disease.Prevalence of DCM is unknown, as there are still no clear criteria for its diagnosis, difficult to carry out epidemiological studies. The incidence according to different authors varies from 5 to 10 per 100 000 population per year. DCM is 2-3 times more common in men, especially those aged 30-50 years.EtiologyIn most cases the etiology is unknown cardiomyopathy (idiopathic form of DCM). Approximately 20% of patients the disease is associated with a history of previous infectious myocarditis. It is believed that exposure of myofibers persisting infectious agents in the body (primarily enteroviruses), including the insertion of viral RNA in the genetic apparatus of the cardiomyocytes or effect "running" viruses autoimmune process leads to damage and mitochondrial disorders of energy metabolism of cells.When using the modern molecular biological technology, especially the polymerase chain reaction (PCR), some patients can not detect the presence of Enterobacteriaceae, in particular, Coxsackie B virus, hepatitis C virus, herpes, cytomegalovirus, etc. The origin of the DCM is also important genetic predisposition to disease. Family cardiomyopathy occurs in aboutcases. They are characterized mainly by autosomal dominant inheritance.Thus, in the genesis of DCM is set to the interaction of several factors: - a genetic predisposition to the disease; - actions of exogenous factors (viral infection, alcohol) and - autoimmune disorders. Remember1. Appearance DCM associated with the interaction of multiple pathogenic factors: - a genetic predisposition to the disease; - actions of exogenous factors (viral infection, alcohol abuse); - autoimmune disorders. 2. Approximately 20% of patients with dilated cardiomyopathy associated with a viral infection. However, in most cases diagnosed idiopathic form of DCM, when reacting these etiological factors becomes so uncertain, that select one of them as the main is not possible.PathogenesisAs a result of the above, and some others (in particular still unknown) etiological factors primary injury occurs cardiomyocytes, reduced energy metabolism of the cells and reduction of myofibrils normally functioning. This leads to a progressive decrease in myocardial contractility and cardiac pump functionI. As a result, reduce the inotropic function of the myocardium occurs dilatation of the heart cavities. Tachycardia (activation CAC), at the same time developing, also helps to maintain cardiac output.For establishment of an adequate pressure in the cavity wall of the dilated ventricle LV should develop much greater intramyocardial tension. The consequences of such a progressive increase in afterload are:- the development of compensatory myocardial hypertrophy ventricles, which contributes some reduction intramyocardial pressure; - an increase in myocardial oxygen demand, which eventually leads to the development of relative coronary insufficiency and myocardial ischemia; - further damage to cardiomyocytes and further reduction in contractility; the development of diffuse and focal kardiofibrozis. The result is a critical reduction of the pumping function of the heart develops expressed myogenic dilatation of the heart chambers.These events reinforced the development of the relative failure of the mitral and tricuspid valves, for even greater dilation of the atria and ventricles. In other words, it is formed and rapidly progressive congestive heart failure (CHF) with a primary function violation ventricular systole, blood stasis in the small and large circles circulation fall in cardiac output and impaired perfusion of the peripheral organs and tissues.It should be noted that a significant increase in heart weight due myocardial hypertrophy Conventional not accompanied by a marked thickening of the ventricular wall, since in DCM always prevails marked dilatation of the heart chambers, which, as it eliminates the increase in muscle mass. Remember1. On the basis of formation of DCM is the primary damage and death of cardiomyocytes hemodynamic consequences are: - the progressive reduction of the contractile ability;- marked dilatation of the heart cavities;- the development of compensatorymyocardial hypertrophy and increasing heart weight (without thickening of ventricular walls) - development, in severe cases of the disease relative insufficiency of the mitral and tricuspid valves;- congestion in the small and large circulation- relative coronary insufficiency and the development of myocardial ischemia; - the development of diffuse and focal myocardial fibrosis;- peripheral vasoconstriction and impaired perfusion of the peripheral organs and tissues.2. The process of cardiac remodeling, and also describes the hemodynamic disorder largely caused by excessive activation of neurohormonal systems of the body 3. The progressive damage to the heart muscle and neurohormonal activation contribute to part of the development in patients with dilated cardiomyopathy:- disturbances of heart rhythm and conduction- intracardiac thrombosis and thromboembolic complications.ClinicClinically, dilated cardiomyopathy is the three main syndromes: 1. systolic heart failure (left ventricular or biventricular) with signs of blood congestion in small and large circulation. 2. A frequent occurrence of rhythm and conduction disturbances (ventricular arrhythmia, atrial fibrillation, atrioventricular block and bundle branch block). 3. thromboembolic events (pulmonary embolism and embolism in an artery of the systemic circulation). complaintsPatients complain of shortness of breath during exercise and at rest, increasing the patient in a horizontal position (orthopnea). In cases in which there are far advanced asthma attacks, often develops at night (cardiac asthma and pulmonary edema). Characteristic of fatigue, muscle weakness, heaviness in the legs during physical stress. Signs of right heart failure (swelling of the legs, heaviness in the right upper quadrant, abdominal distension, dyspepsia) occur later.RememberThe most important clinical feature of the DCM is the rapid and steady progress of the disease and symptoms of decompensation and heart failure refractory to conventional treatment. Patients develop a variety of rhythm and conduction disturbances, some of which cause patients discomfort DCM (palpitations, irregular heart function, paroxysmal tachycardia and atrial fibrillation).The most serious complications are thrombosis and thromboembolism occurs in 20% of patients with dilated cardiomyopathy. Most often, these complications occur in patients with atrial fibrillation.Sometimes patients DCM noted pain in the heart, which in most cases differ from typical angina. Pain often have atypical localization and, as a rule, are not related to physical activity.Physical examination Physical findings obtained in patients with dilated cardiomyopathy, is also very non-specific. At the general examination revealed well-known signs of heart failure: akrozianoz, swelling of the legs, the position orthopnea, abdominal distension, swelling of the neck veins, sometimes positive venous pulse, and others.In the study of light in the lower parts can be auscultated wet finely wheezing. Marked liver enlargement. In cases very advanced, there are signs of cardiac cachexia.Inspection, palpation and percussion of the heart Apical impulse is usually amplified, diffused and moved to the left and down. Most often, it is also possible to detect the amplified and spilled cardiac impulse and epigastric pulsation, which indicates the presence of hypertrophy and dilatation of the RV.It is usually shift to the left of the left border of the relative dullness of the heart and to the right of the right boundary. Dilation LA accompanied by a shift up the top of the heart border and smoothing the "waist" of the heart. Diagnosticselectrocardiography Electrocardiographic examination does not reveal the specific features of the heart electric field characteristic of DCM. usually determined on the ECG:- signs left ventricular hypertrophyand the LA, sometimes in combination with hypertrophy of the RV. Especially characteristic depression RS-T segment in the left chest leads (V5 and V6) and leads I and AVL;signs of left bundle branch block (a common finding) - atrial fibrillation and / or other cardiac arrhythmias; - elongation Q-T interval. EechocardiographyMost typical echocardiographicDCM is significant signs of LV dilation at normal or a reduced thickness of its walls and reduced the PV (below 30-20%). Often marked by the expansion of other chambers of the heart (RV, LA). Usually develop hypokinesia total LV wall, and a significant decrease in blood flow in the ascending aorta and left ventricle introducing path and LA (Doppler). Often rendered intracardiac mural thrombus.However, it should be remembered that in some cases, DCM can detect violations of regional contractility of LV aneurysm and even the tops of the left ventricle, complicates the differential diagnosis of the disease with coronary artery disease.Radiography X-ray examination reveals the following changes: - signs of cardiomegaly; - smoothed contours of the left heart; - spherical shape of the heart due to dilatation of the cavities (in severe cases); - signs of venous and arterial pulmonary hypertension and lung expansion roots. Coronary angiography and ventriculography These invasive research methods are usually used in cases when there is a need for differential diagnosis of dilated cardiomyopathy and coronary heart disease, in particular when deciding on surgical treatment. Endomyocardial biopsy Endomyocardial biopsy is performed in specialized research centers. The method allows to evaluate the degree of destruction of the muscle filaments in biopsy material that has some predictive value.TreatmentTreatment of patients with dilated cardiomyopathy is a very difficult task. Since the cause of the disease in most cases can not be established, pathogenetic therapy patients should be directed to:- CHF correction; - treatment and prevention of arrhythmias; - the treatment and prevention of thromboembolic complications. Modern treatment of chronic heart failure in patients with dilated cardiomyopathy is based on the following principles:1. Restriction of physical activity, as well as salt intake, especially in the presence of edematous syndrome.2. ACE inhibitors is a means of first choice in the treatment of patients with dilated cardiomyopathy. The purpose of these preparations (in the absence of contraindications) advisable at all stages of the disease, even in the absence of marked clinical manifestations of heart failure. ACE inhibitors can prevent necrosis of cardiomyocytes development kardiofibrozis promote regression of hypertrophy, reduce the degree of mitral regurgitation, pressure in LA. Under the influence of the regular prolonged ACE inhibitor improves quality of life in patients with dilated cardiomyopathy. It is proved that the use of ACE inhibitors significantly prolong life in patients with dilated cardiomyopathy.Apply ACE inhibitors patients with dilated cardiomyopathy with caution mainly because of the possibility of hypotensive reactions and emergence of orthostatic hypotension. The initial dose should be minimal:- Enalapril 2.5 mg 2 times a day; - ramipril 1.25 mg 1 time per day - perindopril 2 mg 1 time per day, etc. With good tolerance dose should be increased (corresponding to 20-40 mg per day for enalapril, ramipril 10 mg to 4 mg and for perindopril, etc.). 3. b-blockers is useful to combine with ACE inhibitors. Especially shows b-blockers in patients with stable sinus tachycardia, as well as in patients with atrial fibrillation (possibly in conjunction with cardiac glycosides).Patients with DCM b-blockers are used not only as a means of prevention and treatment of cardiac arrhythmias and heart rate monitor. Their impact on the system is accompanied by improved hemodynamics, reduction of myocardial ischemia and damage to the cardiomyocytes. As well as the ACE inhibitors, b-blockers improve the quality of life, prognosis and survival of patients with dilated cardiomyopathy.Use any b-blockers (metoprolol, bisoprolol, atenolol, carvedilol, etc.). Treatment also begin with low doses of drugs, gradually increasing to their maximum tolerated.The use of b-blockers in DCM requires caution, especially in patients with III-IV heart failure of NYHA FC, although in principle they are not contraindicated, and in severe decompensation. 4. Diuretics are used in the presence of pulmonary congestion and / and in the systemic circulation. In the presence of pronounced edema syndrome advisable listed diuretics combined with the appointment of aldosterone antagonists (aldactone, veroshpiron).5. Nitrates. For the treatment of patients with chronic left ventricular failure as an additional drug can be used nitrates, isosorbide dinitrate or isosorbide-5-mononitrate. The latter are characterized by high bioavailability and predictability of the action (Olikard, imdur et al.). These medicines contribute Escrow blood in the venous line with the systemic circulation, reduce the size of preload and features of pulmonary congestion.6. Cardiac glycosides. These drugs are undoubtedly indicated in patients with persistent atrial fibrillation. In these cases, the beneficial effects of cardiac glycosides (decrease heart rate) are explained mainly positive inotropic action of these drugs and their vagotropic effect, increasingrefractory periodsAV node and deceleration of the electric pulse on the AV node. As a result of atrial fibrillation tachysystolic form fails to translate normosistolichnu that improves diastolic filling processes, reduces the pressure in the LP and veins of the pulmonary circulation and reduces breathlessness and other manifestations of congestion in the lungs.Controversial is the question of the appropriateness of cardiac glycosides in patients with dilated cardiomyopathy sinus rhythmand severe left ventricular systolic dysfunction (ejection fraction 25-30%).The risk of side effects of cardiac glycosides is further increased in the presence of hypokalemia, hypomagnesemia, chronic renal failure, as well as in elderly patients. Thus, the use of cardiac glycosides in critically ill patients with severe left ventricular systolic dysfunction and sinus rhythm, Although it is possible, but only in combination with ACE inhibitors, diuretics, and always under the control of the content of electrolytes and ECG monitoring. Prolonged use of non-glycoside inotropic agents in patients with dilated cardiomyopathy and heart failure is not recommended as significantly increases the mortality rate of these patients. Short-term use non-glycoside inotropic drugs (levodopa, dobutamine, milrinone, amrinone, etc.) is justified only in some cases, for example, in preparing patients for heart transplantation.7. The use of antiplatelet agents shown in all patients DCM as 30% of cases of the disease is complicated by intracardiac thrombosis and thromboembolism. For this purpose a constant acetylsalicylic acid in a dose 0.25-0.3 g per day or using other schemes antiplatelet agents (Trental, dipyridamole, vazobral, Tonakan, Wessel Due F et al.).In patients with atrial fibrillation shows the assignment of indirect anticoagulants (warfarin), coagulation parameters under strict control. Doses are selected to INR value was 2.0-3.0 units.Many experts believe indication for anticoagulants echocardiographic identification of patients with dilated cardiomyopathy intracardiac thrombi. 8. Heart Transplantation. This operation is a highly effective way of treating patients with dilated cardiomyopathy who are refractory to medical therapy. The indications for transplantation are:- rapid progress of heart failure in patients with dilated cardiomyopathy and no effect of the therapy; - the occurrence of life-threatening heart rhythm disorders; - high risk of thromboembolic complications. 10-year survival of patients with dilated cardiomyopathy who underwent a heart transplant operation, as high as 70%. It is clear that a major problem limiting the spread of this form of treatment, is, above all, the high cost of surgery and some organizational problems.9. The two-chamber cardiac electrical stimulation using an implantable pacemaker in some cases, allows for improvement of intracardiac hemodynamics, increasing the function of ventricular systole, and prevent some complications of the disease.Forecast.In general, DCM is characterized by a very poor prognosis. Within 5 years of dying up to 50% of patients, most of them suddenly due to ventricular fibrillation. Other causes of deaths include progressive heart failure and thromboembolic complications.RememberIt should be noted that in recent years a contingent of patients with DCM has changed significantly increased the number of patients with preclinical and subclinical manifestations of the disease. This is due to the widespread introduction into clinical practiceechocardiographicresearch method, the disease allows for early diagnosis. According 1995., The 5-year survival rate of this group of patients was 76% DCM. Operation heart transplantation significantly improves the prognosis of patients with dilated cardiomyopathy: a 10-year survival rate is 70%.Restrictive cardiomyopathyRestrictive cardiomyopathy (RCM) is a heterogeneous group of primary (idiopathic) or secondary heart diseases involving lesions endocardial and / or the myocardium, which leads to a pronounced fixed restriction filling of the ventricles during diastole and development of diastolic dysfunction of one or both ventricular diastole and progressing CHF. EtiologyDistinguish primary idiopathic RCM, the etiology of which is unknown, and secondary restrictive heart disease, developing in some well-known diseases. The causes of restrictive cardiomyopathyPrimary (idiopathic) forms RCMSecondary forms of RCMendomyocardial fibrosis Amyloidosis (preferably primary) Fibroplasticendocarditis (Loeffler's disease) hemochromatosis systemic scleroderma sarcoidosis Carcinoid heart disease glycogenoses Radiation damage to the heart The defeat of the cardiac conduction system (Fabry disease) The primary (idiopathic) form of RCM currently carry two diseases: - endomyocardial fibrosis; - Fibroplastic endocarditis (Loeffler's disease). PathogenesisCommon features to all these diseases, the underlying formation RCM is expressed limitation (restriction) filling of the ventricles during diastole and gradual decrease ventricular cavity sizes, up to their obliteration. An important feature of the diastolic filling of the ventricles in patients with RCM is that it is carried out at the beginning of diastole during a brief period of rapid filling, and further fill the ventricles with blood almost never happens. At the same time it is increasing the pressure in the atrium and in the veins of small and large stakes circulation. Finally, developing CHF left- right ventricular or biventricular, depending on which heart failure department predominates.RememberAt RCM most characteristic hemodynamic and morphological changes are:1. The marked thickening, induration and decreased elasticity endocardial and / or infarction.2. Restrictive (restrictive) fixed breach the blood filling of one or both ventricles, the filling of the blood is carried out only at the beginning of a period of rapid filling.3. Reducing the ventricular cavity sizes and a simultaneous significant expansion atrium and atrial overload caused by the presence of mitral and tricuspid insufficiency.4. The blood froze in the veins of small and / or large stakes circulation.5. Education intracardiac thromb and an increased risk of thromboembolic complications.ClinicThe clinical picture includes RCM systemic manifestations and symptoms of the underlying disease left- or right ventricular diastole biventricular heart failure. The predominance of lesions of the right heart characterized by signs of right heart failure. Patients appear pronounced swelling, akrozianoz, ascites, congestive liver and spleen, swelling of the neck veins, hepato-jugular reflux and other symptoms.Auscultation determined attenuation I and II heart sounds. At the top and left of the sternum in the IV-V intercostal space usually well auscultated III abnormal heart .Systolic murmur of tricuspid insufficiency is listened in the lower third of the sternum.The predominance of the defeat left heart is accompanied by signs of left ventricular and then biventricular heart failure. Patients first complain of shortness of breath, attacks of breathlessness, weakness, fatigue. Auscultation I determined the weakening tone, accent II tone on the pulmonary artery, a loud tone on pathological III apex of the heart and the systolic murmur, which is held in the left axilla. Over time, these characters join the clinical manifestations of right heart failure (swelling of the feet, cyanosis, hepatomegaly, ascites, jugular venous distention, etc.).Diagnosticselectrocardiography ECG changes at RCM nonspecific. Depending on the nature of the causes of disease and heart disease on the ECG can be detected:- low voltage ventricular complex QRS; - nonspecific changes RS-T segment and T wave; - blockade of left bundle branch block beam (less other intraventricular and atrioventricular block) - abnormal heart rhythm (atrial fibrillation, arrythmia, etc.). - signs of overload of the atria (P-mitrale and P-pulmonale). radiography The x-ray is usually determined by the normal or reduced size of the heart, with the exception of radiological signs of atrial enlargement. Also revealed signs of stagnation of blood in the pulmonary circulationechocardiographyechocardiographyis the main method to suspect the presence of patient RCM.In patients with RCM can be found:1. Thickening endocardial a decrease in the size of the ventricular cavity 2. Various variants of paradoxical movement IVS. 3. prolapsed mitral and tricuspid valves.4. The relative insufficiency of the mitral and tricuspid valves. 5. The presence of intracardiac mural thrombus. TreatmentIn most cases, medication is ineffective RCM.In the event of heart failure diuretics (hydrochlorothiazide, furosemide), vasodilators (nitrates), oral anticoagulants, although it is difficult to count on the good effects of such therapy. Moreover, the use of diuretics, nitrates, and can substantially reduce venous return to the heart, which further limits the filling of the ventricles and the cardiac output.The use of cardiac glycosides when RCM also not shown as myocardial function and ventricular systole in most cases are normal, and factor limiting the circulation, is a disturbance of filling ventricular diastole. When there is fibroplastic type possible surgical treatment. The operation consists of excision of the thickenedendocardial and restoring impaired ventricular diastolic function. ForecastWhen the clinical signs of heart failure prognosis is very unfavorable: of patients die within 1.5-2.0 years. Forecast worsens further in the event of arrhythmias and thromboembolic complications. Operative treatment of disease Leffler in some cases, significantly improves the condition of patients and the prognosis of the disease.Methodical recommendation is made bydoc. Pavlovskaya E.N, ass. Ryabinina A.H ................
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