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 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 gradient2- ECG: LVH & ST and T wave changes

Treatment :1. Beta blockers to reduce LV contractility

and thus reduce the outflow obstruction2. Verapamil : that improve the diastolic

function3. Amiodarone : anti-arrhythmic drug4. 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 dysfunction2. Endomyocardial biopsy. Treatments : small doses of diuretic