CARDIOMYOPATHIES

notes Dr. Italian Claudio

. We are making reference to diseases of the heart muscle is not determined by:

made ischemic

valvular

hypertension, pulmonary / systemic

diseases of the pericardium

CARDIOMYOPATHIES PRIMITIVE:

Or hypertrophic cardiomyopathy, with no obstruction

Ø dilated cardiomyopathy or congestive

Ø restrictive cardiomyopathy obliterative



In this website we will deal specifically dilated or congestive form, characterized by enlargement of cardiac chambers with low fraction of pump (ejection fraction)



Dilated cardiomyopathy (congestive) (CMD)

This form of heart disease of unknown etiology, is characterized, as we said, for a reduction in force of contraction of the heart (impaired ventricular systolic function), together with altered left ventricular relaxation (impaired ventricular diastolic function), then not only the heart does not pump, but also fails to fill as it would be appropriate for. So this results in a low cardiac output. Typical is the cardiomegaly with dilatation of both ventricles, decreased ejection fraction and increased end-diastolic volume. The cardiac output decreased (anterograde deficit) and you come to a stasis of blood to the heart of the upstream (retrograde deficit), with clear "air hunger of the patient" (cf. dyspnea, heart failure).

Epidemiology. It 's the most common form of cardiomyopathy primitive, so that its incidence was estimated to be approximately 5 cases per 100,000 person-years, especially in middle-aged males, M: F = 2-3:1 in the 10 - l5% incidence of cases with family (other than genetic alterations, eg. in the gene for dystrophin).

Etiology: In most cases unknown, it seems it may be caused by:

Ø 1 / 3 of cases is probably the result of an enterovirus myocarditis. In fact, myocardial cells can be found in the DNA of Enterovirus and talk of a viral genesis posmiocardite.

Or other patients are elements suggesting an autoimmune pathogenesis, eg. anti-β1-adrenergic receptor cells of my heart, namely Members of those receptors to nerve transmission of signals.



Pathological anatomy: marked dilatation biventricular hypertrophy in the presence of moderate and often parietal thrombus formation (source of embolism and pulmonary embolism!).

Histology: thin myocytes with enlarged nuclei, with bizarre shapes, reduction of myofibrils, presence of myelin.

There are 3 types according to the size of interstitial fibrosis:

Ø Type A non fibrotizzante

Or type B: fibrotizzante widespread

Or type C: fibrotizzante localized.
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Clinical: left heart failure with progressive dyspnea on exertion (heart failure as a result of global), rhythm disturbances (arrhythmias especially ventricular type).

Complications consist of pulmonary embolism and arterial thrombus formation for cardiac arrhythmias and sudden death.



Diagnosis

The workshop demonstrates the presence of anti-miolemma.

Chest radiography: cardiomegaly and signs of pulmonary stasis.

Echocardiography appears biventricular dilatation (left atrium even in case of failure

on mitral), decreased range of motion (hypokinesia) of the ventricular wall with systolic anterior motion of its limitation. The index of the reduced contractility is reduced (<30%) of fractional shortening, which is roughly the angiographic ejection fraction. Often they are demonstrable

thrombi in the ventricle and / or atrium (best viewed with transesophageal ultrasound.

Invasive diagnostics

- Biopsy myocardial histology + / Immunohistological / diagnostic virological

Differential Diagnosis

CMD-secondary education, for example. from toxic factors (alcohol, adriamycin, anthracycline, mitoxantrone, tricyclic antidepressants and other drugs).

- Viral myocarditis (in a natural evolution of patients).

Diagnosis: clinical - Echocardiography - myocardial biopsy - removal of a secondary cardiomyopathy.



Therapy

- Abstaining from alcohol (see, suspension of cardiotoxic drugs

- + Standard therapy for heart failure treatment, physical rest, ACE inhibitors, diuretics, digital. About 2 / 3 of the patients also benefit from the administration of β-blockers (study with etoprololo MDC), since they also argue, however, episodes of heart failure, the β-blockers should be administered

only in the context of controlled trials in low starting doses, eg. carvedilol.

- Prophylaxis of thromboembolism with anticoagulants

- In case of threatening ventricular arrhythmias, air cardiovertitore / defibrillator

- In clinical trials in the context of controlled trials:

. In proven cases of autoimmune response and in the absence of documentation of viral cause, evtl. immunosuppressive therapy

. in the case of documented presence of viral RNA, evtl. antiviral therapy

- Terminal heart failure attempt to "relief" by heart

temporary mechanical heart replacement

- <LAST Ratio>: cardiac transplantation.

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