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: Diastolic dysfunction Due to - LV obstruction. - arrhythmias

 Signs : 1. Jerky pulse (the pulse rapidly but stop suddenly). 2. Palpable & audible 4 th 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 reduce the outflow obstruction 2. Verapamil : that improve the diastolic function 3. Amiodarone : anti-arrhythmic drug 4. Surgical excision : of part of the bulging interventricular septum. 5. 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