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Cardiomyopathies

Cardiomyopathies. Dr. Hesham K. Rashid, MD Ass. Professor of Cardiology Benha University. Definition : it is a disease of heart muscle of unknown etiology. Types : 1. Dilated myopathy 2. Hypertrophic myopathy . 3. Restrictive myopathy. Dilated myopathy. Etiology :

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Cardiomyopathies

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  1. Cardiomyopathies Dr. Hesham K. Rashid, MD Ass. Professor of Cardiology BenhaUniversity

  2. Definition : it is a disease of heart muscle of unknown etiology. • Types : 1. Dilated myopathy 2. Hypertrophic myopathy. 3. Restrictive myopathy

  3. Dilated myopathy • Etiology : • Primary : of unknown cause. • Secondary : - generalized disease involving myocardium as systemic lupus , beriberi , muscular dystrophies, endocrine disorders as acromegaly. - secondary to toxic effect of alcohol. - secondary to toxic drugs as anticancer adriamycin - puerperal cardiomyopathy occurs in late pregnancy. - ischemic myopathy due to infarction or ischemia

  4. Effect CLINICAL SYMPTOMS & SIGNS OF HEART FAILURE

  5. Clinical picture : • Symptoms & signs of LV failure. • Symptoms & signs of RV failure. • Symptoms & signs of both LV & RV

  6. Investigations 1- plain X-ray : - cardiomegally. - lung congestion

  7. 2- ECG : - Sinus tachycardia. - LBBB. - Arrhythmias

  8. 3-Echocardiography • Dilated LV . • Impaired LV systolic functions • Global hypokinisia • LV thrombus can be seen

  9. Treatments : • Treatment of underlining causes. • Anti-failure treatment: - Rest & salt restriction. - Diuretics. - ACE inhibitor. - Digitalis. - Oral anticoagulant - Anti-arrhythmic drugs. • Cardiac transplantation.

  10. Hypertrophic cardiomyopathy • Etiology : most cases are inherited as autosomal dominant , some cases are sporadic • Pathophysiology : - disarray of cardiomyocytes ( inappropriate hypertrophy of the myocardium ) leading to : 1- Ischemia 2- Arrhythmias 3- diastolic dysfunction - asymmetric septal hypertrophy (ASH). - LV outflow tract obstruction during systole. - systolic anterior motion of mitral valve (SAM). - Small LV with excellent systolic function.

  11. SAM ASH

  12. Due to : -Increase o2 consumption - Hypertrophy of ms.kink on coronary artery Due to - LV obstruction. - arrhythmias Due to: Diastolic dysfunction

  13. Signs : • Jerky pulse (the pulse rapidly but stop suddenly). • Palpable & audible 4th heart • Systolic murmur due to : - LV outflow obstruction at left sternal border that increased with standing position or valsalva - mitral regurge at the apex from abnormal mitral valve

  14. Investigations 1- Echocardiography: * ASH * SAM * Thick LV with small cavity. * Excellent systolic function with marked impaired diastolic function * variable dynamic pressure gradient 2- ECG: LVH & ST and T wave changes

  15. Treatment : • Beta blockers to reduce LV contractility and thus reduce the outflow obstruction • Verapamil : that improve the diastolic function • Amiodarone : anti-arrhythmic drug • Surgical excision : of part of the bulging interventricular septum . • Injection of alcohol in coronary artery which supplies the IVS causing its infarction & atrophy.

  16. Restrictive myopathy • It occurs when myocardium becomes less compliance and thus more difficult to distend and fill making increase in the atrial pressure ( severe diastolic dysfunction ). • Etiology : infiltrative diseases of the myocardium as amyloidosis , scleroderma , hemochromatosis.

  17. Symptoms : • Pulmonary congestive symptoms. • Systemic congestive symptoms • Signs : • Congestive neck vein. • Fourth heart sounds. • Investigations : • Echocardiography : diastolic dysfunction • Endomyocardial biopsy. • Treatments : small doses of diuretic

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