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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 : 1. Primary : of unknown cause. 2. 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 Effect CLINICAL SYMPTOMS & SIGNS OF HEART FAILURE Clinical picture : Symptoms & signs of LV failure. Symptoms & signs of RV failure. Symptoms & signs of both LV & RV Investigations 1- plain X-ray : - cardiomegally. - lung congestion 2- ECG : - Sinus tachycardia. - LBBB. - Arrhythmias 3-Echocardiography •Dilated LV . •Impaired LV systolic functions •Global hypokinisia •LV thrombus can be seen Treatments : Treatment of underlining causes. Anti-failure treatment: - Rest & salt restriction. - Diuretics. - ACE inhibitor. - Digitalis. - Oral anticoagulant - Anti-arrhythmic drugs. Cardiac transplantation. 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. SAM ASH Due to : -Increase o2 consumption - Hypertrophy of ms.kink on coronary artery Due to - LV obstruction. - arrhythmias Due to: Diastolic dysfunction Signs : 1. Jerky pulse (the pulse rapidly but stop suddenly). 2. Palpable & audible 4th heart 3. 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 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 Treatment : 1. Beta blockers to reduce LV contractility and thus 2. 3. 4. 5. 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. 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. Symptoms : 1. Pulmonary congestive symptoms. 2. Systemic congestive symptoms Signs : 1. Congestive neck vein. 2. Fourth heart sounds. Investigations : 1. Echocardiography : diastolic dysfunction 2. Endomyocardial biopsy. Treatments : small doses of diuretic