cardiomyopathy dr. meg-angela christi m. amores. cardiomyopathy a condition primarily nvolving the...
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Cardiomyopathy
Dr. Meg-angela Christi M. Amores
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Cardiomyopathy
• a condition primarily nvolving the myocardium• not the result of congenital, acquired valvular,
hypertensive, coronary arterial, or pericardial abnormalities
• 2 forms:– Primary type– Secondary type
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Primary cardiomyopathy
• consisting of heart muscle disease predominantly involving the myocardium and/or of unknown cause
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Secondary cardiomyopathy
• consisting of myocardial disease of known cause or associated with a systemic disease such as amyloidosis or chronic alcohol use (
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three morphologic types
• dilated, restrictive, and hypertrophic
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three morphologic types
• 1. Dilated: Left and/or right ventricular enlargement, impaired systolic function, congestive heart failure, arrhythmias, emboli
• 2. Restrictive: Endomyocardial scarring or myocardial infiltration resulting in restriction to left and/or right ventricular filling
• 3. Hypertrophic: Disproportionate left ventricular hypertrophy, typically involving septum more than free wall, with or without an intraventricular systolic pressure gradient; usually of a nondilated left ventricular cavity
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Dilated cardiomyopathy
• 1/3 of all cases• LV and/or right ventricular (RV) systolic pump function
is impaired, leading to progressive cardiac dilatation (remodeling)
• Symptoms of heart failure (HF) typically appear only after remodeling has been ongoing for some time (months or even years)
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Dilated cardiomyopathy
• In most cases, no cause is apparent• Familial• end result of myocardial damage • may be the late consequence of acute viral
myocarditis • most commonly becomes apparent clinically
in the third or fourth decades• reversible form of DCM may be found with
alcohol abuse, thyroid disease, cocaine use and chronic uncontrolled tachycardia
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Dilated cardiomyopathy
CLINICAL FEATURES• Symptoms of left- and right-sided CHF usually
develop gradually • vague chest pain may be present• Syncope due to arrhythmias and systemic
embolism (often emanating from a ventricular thrombus) may occur
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Dilated cardiomyopathy
PHYSICAL EXAMINATION• Variable degrees of cardiac enlargement • findings of Congestive heart failure
• rales, edema
• pulse pressure is narrow • jugular venous pressure is elevated
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Dilated cardiomyopathy
LABORATORY• Chest Xray– enlargement of the cardiac silhouette due to LV
dilatation– pulmonary vascular redistribution – alveolar edema
• ECG– Sinus tachycardia,atrial fibrillation, ventricular
arrhythmias, left atrial abnormality, low voltage
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Treatment
• Most patients die within 4 years• ¼ have spontaneous improvement• patients should be considered for chronic
anticoagulation• Standard therapy for heart failure• Alcohol should be avoided - cardiotoxic
effects
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Hypertrophic cardiomyopathy
• characterized by LV hypertrophy, typically of a nondilated chamber, without obvious cause
• 1 in 500 of the general population• pathophysiologic abnormality is diastolic
dysfunction
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Hypertrophic cardiomyopathy
• majority demonstrate a ventricular septum whose thickness is disproportionately increased when compared with the free wall
• half of all patients with HCM have a positive family history
• genetic testing may allow a definitive diagnosis of HCM
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Hypertrophic cardiomyopathy
CLINICAL FEATURES• Variable• asymptomatic or mildly symptomatic • first clinical manifestation may be SCD (sudden
cardiac death)• occurring in children and young adults during or after
physical exertion• most common cause of SCD in young competitive
athletes
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Hypertrophic cardiomyopathy
PHYSICAL EXAMINATION• hallmark of obstructive HCM is a systolic
murmur, which is typically harsh, diamond-shaped, and usually begins well after the first heart sound
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Hypertrophic cardiomyopathy
LABORATORY• ECG:
• LV hypertrophy and widespread deep, broad Q waves
• Chest Xray• May be normal or mild increase in silhouette
• Echocardiogram• Mainstay in diagnosis• LV hypertrophy, often with the septum 1.3 times the
thickness of the posterior LV free wall
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Hypertrophic Cardiomyopathy
TREATMENT• Dehydration avoided• Diuretics used in caution• Amiodarone appears to be effective in
reducing the frequency of supraventricular as well as of life-threatening ventricular arrhythmias
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Restrictive Cardiomyopathy
• abnormal diastolic function • walls are excessively rigid and impede ventricular
filling• Myocardial involvement with amyloid is a
common cause of secondary restrictive cardiomyopathy
• transplanted heart, in hemochromatosis, glycogen deposition, endomyocardial fibrosis, sarcoidosis, hypereosinophilic disease, and scleroderma
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Restrictive Cardiomyopathy
CLINICAL FEATURES– inability of the ventricles to fill limits cardiac
output and raises filling pressures– exercise intolerance and dyspnea – dependent edema, ascites, and an enlarged,
tender, and often pulsatile liver– heart sounds may be distant, and third and fourth
heart sounds are common
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Restrictive Cardiomyopathy
LABORATORY• ECG – low-voltage, nonspecific ST-T-wave abnormalities
and various arrhythmias• Echocardiography, CTI, and CMRI – symmetrically thickened LV walls and normal or
slightly reduced ventricular volumes and systolic function