CARDIOMYOPATHY

Abraha Hailu, MD

Etiology and classification
• Cardiomyopathies:
 Are group of diseases that primarily affect the heart muscle and are not the result of congenital, acquired valvular, hypertensive, coronary arterial, or pericardial abnormalities

• Two fundamental forms:
 primary type: disease predominantly involving the myocardium and/or of unknown cause  secondary type: myocardial disease of known cause or associated with a systemic disease such as amyloidosis or chronic alcohol use
 In many cases it is not possible to arrive at a specific etiologic diagnosis, and thus it is often more desirable to classify the cardiomyopathies into one of three morphologic types (dilated, restrictive, and hypertrophic) on the basis of differences in their pathophysiology and clinical presentation
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ClassificationandCardiomyopathies Etiology of classification
• Primary Myocardial Involvement:
 Idiopathic (D,R,H)  Familial (D,R,H)  Eosinophilic endomyocardial disease (R)  Endomyocardial fibrosis (R)

• Secondary Myocardial Involvement:
 Infective (D):
 Viral myocarditis  Bacterial myocarditis  Fungal myocarditis  Protozoal myocarditis  Spirochetal , Rickettsial
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D):  Amyloidosis  Sarcoidosis  Malignancy 3/29/2012 ABRAHA HAILU 4 .R)  Glycogen storage disease  Mucopolysaccharidoses  Hemochromatosis  Connective tissue disorders (D):  Systemic lupus erythematosus  Polyarteritis nodosa  Rheumatoid arthritis  Progressive systemic sclerosis  Dermatomyositis  Infiltrations and granulomas (R.Etiology and classification  Familial storage disease (D.

Etiology and classification Neuromuscular (D)  Muscular dystrophy  Myotonic dystrophy Friedreich's ataxia (H.D) Sensitivity and toxic reactions (D)  Alcohol  Radiation  Drugs Peripartum heart disease (D) 3/29/2012 ABRAHA HAILU 5 .

Clinical Classification of Cardiomyopathies • Dilated CMP:  Left and/or right ventricular enlargement.  arrhythmias.  Emboli • Restrictive CMP:  Endomyocardial fibrosis/scarring or myocardial infiltration resulting in restriction to left and/or right ventricular filling • Hypertrophic CMP:  Disproportionate left ventricular hypertrophy.  congestive heart failure. with or without an intraventricular systolic pressure gradient. usually of a nondilated left ventricular cavity 3/29/2012 ABRAHA HAILU 6 . typically involving septum more than free wall.  impaired systolic function.

Clinical Classification of Cardiomyopathies 3/29/2012 ABRAHA HAILU 7 .

and chronic uncontrolled tachycardia 3/29/2012 ABRAHA HAILU 8 . pregnancy.Dilated Cardiomyopathy • LV and/or RV systolic pump function is impaired. or toxic agents • may occur at any age. but most commonly becomes apparent clinically in the third or fourth decades • reversible form of DCM may be found with alcohol abuse. thyroid disease. leading to progressive cardiac dilatation  Symptoms of heart failure typically appear only after remodeling has been ongoing for some time (months/yrs) • is either familial or the end result of myocardial damage produced by a variety of known or unknown infectious. cocaine use. metabolic.

and right-sided CHF usually develop gradually • vague chest pain may be present.Clinical Features • Symptoms of left. and mitral or tricuspid regurgitation may occur 3/29/2012 ABRAHA HAILU 9 . typical angina pectoris is unusual and suggests the presence of IHD • Syncope due to arrhythmias and systemic embolism may occur • Physical Examination:  Variable degrees of cardiac enlargement  In patients with advanced disease. the pulse pressure is narrow and the jugular venous pressure is elevated  Third and fourth heart sounds are common.

alveolar edema • ECG:  shows sinus tachycardia or atrial fibrillation. left atrial abnormality. ventricular arrhythmias. minimally thickened. CT show LV dilatation. in advanced cases. and sometimes intraventricular and/or AV conduction defects • Echocardiography. and systolic dysfunction • Circulating levels of BNP are usually elevated 3/29/2012 ABRAHA HAILU 10 . or thinned walls. low voltage. with normal.Laboratory Examinations • CXR:  enlargement of the cardiac silhouette due to LV dilatation or generalized cardiomegaly  The lung fields may demonstrate pulmonary vascular redistribution and interstitial or. Cardiac MRI. diffuse nonspecific ST-T-wave abnormalities.

or bradyarrhythmia • Systemic embolization is a concern.Prognosis and treatment • Patients of African ancestry are more likely to suffer rapidly progressive CHF and death than Caucasians • Death is due to either progressive HF or ventricular tachy. and patients should be considered for chronic anticoagulation • Standard therapy of HF • Cardiac resynchronization therapy and insertion of an implantable cardioverter defibrillator (ICD)…if indicated 3/29/2012 ABRAHA HAILU 11 .

as well as the DD form of the angiotensin-converting enzyme (ACE) gene increase the predilection for the development of alcoholic cardiomyopathy • Patients with advanced alcoholic cardiomyopathy and severe CHF have a poor prognosis • Management consists of abstention. which may halt the progression or even reverse the course of this disease 3/29/2012 ABRAHA HAILU 12 . alcohol dehydrogenase (ALDH2*2).Alcoholic Cardiomyopathy • Individuals who consume large quantities (>90 g/d) of alcohol over many years may develop a clinical picture resembling idiopathic or familial DCM • The risk of developing cardiomyopathy is partially determined genetically  A polymorphism of the gene encoding the alcohol metabolizing enzyme.

followed by atrial flutter and frequent PVCs 3/29/2012 ABRAHA HAILU 13 .Alcoholic Cardiomyopathy • holiday heart syndrome:  typically appears after a drinking binge  may be found in individuals without overt HF and consists of recurrent supraventricular or ventricular tachyarrhythmias  atrial fibrillation is seen most frequently.

although inflammatory myocarditis. and gestational hypertension have all been incriminated • The patient who develops peripartum cardiomyopathy typically is multiparous. immune activation. and >30 years. although the disease may be found in a wide spectrum of patients • The mortality rate of this disorder is around 10% 3/29/2012 ABRAHA HAILU 14 .Peripartum Cardiomyopathy • cause is unknown. of African ancestry.

and further pregnancies frequently produce additional myocardial damage. subsequent pregnancies may sometimes be tolerated. and/or the LV ejection fraction (EF) remains depressed after 6 months. albeit with an increased risk of recurrent CHF – if the heart remains enlarged. particularly if LV dysfunction persists 3/29/2012 ABRAHA HAILU 15 .Peripartum Cardiomyopathy • The prognosis is related to whether the heart size returns to normal after the first episode of CHF – If it does. the prognosis is poor. ultimately leading to refractory CHF • Patients who recover from peripartum cardiomyopathy should be encouraged to avoid further pregnancies.

hypertension.doxorubicin)… usual culprits • Systolic dysfunction and ventricular arrhythmias occur in a dose-dependent manner with a dose >450 mg/m2 and are frequent with doses >550 mg/m2  The development of these complications appears to be related to damage to the inner mitochondrial membrane and interference with the synthesis of ATP • is related not only to the dose of the drug but also to the presence or absence of several risk factors. underlying heart disease.Anthracycline CMP • anthracycline derivatives (EX. which include cardiac irradiation. and concurrent treatment with cyclophosphamide 3/29/2012 ABRAHA HAILU 16 .

patients may manifest RV failure The ECG typically shows QRS prolongation localized to the right precordial leads CTI and CMRI typically show RV dilatation. RV aneurysm. and fatty replacement 3/29/2012 ABRAHA HAILU 17 .Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia •familial cardiomyopathy characterized by progressive fibrofatty replacement of predominantly the RV myocardium On clinical examination.

Tako-Tsubo Cardiomyopathy • Also known as apical ballooning syndrome • characterized by the abrupt onset of severe chest discomfort preceded by a very stressful emotional or physical event • most common in women >50 years and is accompanied by STsegment elevations and/or deep T-wave inversions in the precordial leads – No obstruction in the epicardial coronary arteries is noted on angiography – There is severe akinesia of the distal portion of the left ventricle with reduction of the EF • Troponins are usually mildly elevated 3/29/2012 ABRAHA HAILU 18 .

is involved • Rx: BB? 3/29/2012 ABRAHA HAILU 19 . acting on the epicardial coronary vessels and/or coronary microcirculation. are reversible within 3–7 days and do not cause longterm cardiac dysfunction or disability • mechanism responsible is not clear.Tako-Tsubo Cardiomyopathy • Cardiac imaging typically shows "ballooning" of the left ventriclar apex in end-systole – these changes. although it is likely that an adrenergic surge that includes circulating catecholamines.

often with preferential hypertrophy of the interventricular septum. such as hypertension or aortic stenosis • Two features of HCM:  asymmetric LV hypertrophy.Hypertrophic Cardiomyopathy • characterized by LV hypertrophy. and  a dynamic LV outflow tract pressure gradient. related to narrowing of the subaortic area • diastolic dysfunction results in elevated LV end-diastolic pressures 3/29/2012 ABRAHA HAILU 20 . typically of a nondilated chamber. without obvious cause.

Hypertrophic Cardiomyopathy • patterns of hypertrophy:  Majority ventricular septum  symmetric hypertrophy.  mid-ventricular cavity  apex or  LV free wall  About half of all patients with HCM have a positive family history 3/29/2012 ABRAHA HAILU 21 .

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and usually begins well after the first heart sound • The murmur is best heard at the lower left sternal border as well as at the apex 3/29/2012 ABRAHA HAILU 23 . angina pectoris.Clinical Features • Many patients are asymptomatic or mildly symptomatic • Unfortunately. and fatigue Physical Examination • a double or triple apical precordial impulse and a fourth heart sound • a systolic murmur. which is typically harsh. diamond-shaped. the first clinical manifestation may be SCD • the most common complaint is dyspnea • Other symptoms include presyncope/syncope.

– decreased ventricular preload. and – decreased aortic impedance and pressure (afterload) 3/29/2012 ABRAHA HAILU 24 .Hemodynamics • Obstruction appears to result from narrowing of the LV outflow tract by systolic anterior movement (SAM) of the mitral valve against the hypertrophied septum • Three basic mechanisms are involved in the production and intensification of the dynamic intraventricular obstruction: – increased LV contractility.

3 times the thickness of the posterior LV free wall  SAM of the mitral valve + MR is found in patients with pressure gradients • CMRI 3/29/2012 ABRAHA HAILU 25 .Laboratory Evaluation • ECG commonly shows LV hypertrophy and widespread deep. broad Q waves or arrhythmias • Echocardiogram:  septum 1.

and certain genetic mutations 3/29/2012 ABRAHA HAILU 26 .  a family history of SCD. ventricular tachycardia on ambulatory monitoring or at electrophysiologic testing.  marked ventricular hypertrophy (ventricular septal thickness >30 mm).• Patients at increased risk of SCD include those with:  a history of resuscitation.  failure of blood pressure to rise during exercise. recurrent syncope.

and anecdotal data suggest that it may reduce the risk of SCD 3/29/2012 ABRAHA HAILU 27 . and diuretics used with caution • B-Adrenergic blockers ameliorate angina pectoris and syncope in 30-50% of patients  these drugs may limit the increase in the gradient that occurs during exercise • Amiodarone appears to be effective in reducing the frequency of supraventricular as well as of life-threatening ventricular arrhythmias.Treatment • competitive sports and very strenuous activities should be avoided • Dehydration should be avoided.

and B-adrenergic agonists are best avoided • Atrial fibrillation is poorly tolerated. and a strong effort should be made to restore and then maintain sinus rhythm • Surgical myotomy/myectomy of the hypertrophied septum  usually abolishes intraventricular obstruction and provides lasting symptomatic improvement in about three-quarters of severely symptomatic patients with large pressure gradients who are unresponsive to medical management • alcohol septal ablation can also reduce obstruction and improve symptoms 3/29/2012 ABRAHA HAILU 28 . nitrates. dihydropyridine calcium blockers. vasodilators.Treatment • diuretics.

sarcoidosis. 3/29/2012 ABRAHA HAILU 29 . hemochromatosis. endomyocardial fibrosis.. hypertrophy. and in neoplastic infiltration….Restrictive Cardiomyopathy • The hallmark is abnormal diastolic function – the ventricular walls are excessively rigid and impede ventricular filling  In late stages systolic function is also impaired • Etiology: Myocardial infiltration. glycogen deposition. sarcoidosis. and scleroderma. or endomyocardial fibrosis due to: – Amyloidosis. hypereosinophilic syndrome. following mediastinal irradiation.

particularly those with substantial concomitant endocardial involvement. partial obliteration of the ventricular cavity by fibrous tissue and thrombus contributes to the abnormally increased resistance to ventricular filling  Thromboembolic complications are frequent in such patients 3/29/2012 ABRAHA HAILU 30 .• In many of these conditions.

Left: Gross specimen. demonstrating prominent biatrial enlargement. with normal-sized ventricles Right: Light microscopy showing marked interstitial fibrosis 3/29/2012 ABRAHA HAILU 31 .

ascites.Clinical Features • The inability of the ventricles to fill limits cardiac output and raises filling pressures. exercise intolerance and dyspnea are usually prominent  these patients commonly have dependent edema. tender. and mitral regurgitation is more common  Differentiation of RCM from constrictive pericarditis is of importance b/c the latter is often curable by surgery 3/29/2012 ABRAHA HAILU 32 . thus. and often pulsatile liver • The jugular venous pressure is elevated and does not fall normally with inspiration (Kussmaul's sign) • third and fourth heart sounds are common  In contrast to constrictive pericarditis. the apical impulse is usually easily palpable. and an enlarged.

hypertrophy.…. markedly enlarged atria • Biopsy: Fibrosis. infiltration Constrictive pericarditis • Hx of acute pericarditis. radiation Rx. sarcoidosis…. • CXR: no pericardial calcification • ECG: BBB. cardiac surgery.Features that help to distinguish RCMP from constrictive pericarditis RCMP • Hx of systemic diseases that involve the myocardium: amyloidosis. AV Blocks • CT/MRI: Normal pericardium • Echo: myocardial thickening. • • • • CXR: pericardial calcification present ECG: abnormal repolarization CT/MRI: thick pericardium (>4mm) Echo: normal myocardial thickness and atrial sizes • Biopsy: Normal 3/29/2012 ABRAHA HAILU 33 .

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