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Cardiomyopathy,

Definition

• The term cardiomyopathy is purely


descriptive, meaning disease of the
heart muscle

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Cardiomyopathy

Nursing Review, 2001


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Functional Classification
 Dilated (Congestive) Ventricular dilation, hypokinetic left ventricle,
and systolic dysfunction
 Hypertrophic (HCM,)
 Inappropriate myocardial hypertrophy, with or without left ventricular
obstruction
 Restrictive (Infiltrative)
 Abnormal ventricular filling with diastolic dysfunction

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Dilated Cardiomyopathy

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Dilated Cardiomyopathy -
Definition
• Primary (idiopathic) is a disease of unknown etiology that
principally affects the myocardium leading to LV dilation and
systolic dysfunction

• Secondary causes include ischemia, alcoholic, peripartum, post-


infectious, viral

• Most common of the cardiomyopathies

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Schematic of Dilated CM

Medslides.com
stretchedmerentang
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Clinical Manifestations
• Highest incidence in middle age
– Blacks 2x more frequent than whites
– Men 3x more frequent than women
• Symptoms may be gradual in onset
• Acute presentation
– Misdiagnosed as viral URI in young adults
– Uncommon to find specific myocardial disease on
endomyocardial biopsy

Source: UpToDate (www.uptodate.com) "Clinical manifestations and evaluation of the patient with suspected heart
failure" Wilson S Colucci, MD, last updated 6/7/09

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Diagnostics
 CXR (enlarged heart, CHF)
 Electrocardiogram (tachycardia, A-V block, LBBB, NSSTT changes,
PVC’s)
 24-hour Holter monitor
 if lightheadedness, palpitation, syncope
 Echocardiogram (left ventricular dilation, global hypokinesis, low
EF)
 Myocardial biopsy, rare
 Cardiac catheterization (R/O CAD)
 if age >40, ischemic history, high risk profile, abnormal ECG

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DCM - Treatment
• Limit activity based on functional status
• Salt restriction
• Fluid restriction
• Initiate medical therapy
– ACE inhibitors, diuretics
– Digoxin,
– Hydralazine/nitrate combination
– Anticoagulation prn (EF <30%, hx of embolic events)

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Hypertrophic Cardiomyopathy

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Hypertrophic
Cardiomyopathy (HCM)
• Defined
– First described in 1869 and accepted as a clinical entity in the
1950’s
– Prevalence 2:1000
– Genetic disease characterized by hypertrophy of the left ventricle
with marked variable clinical manifestations morphologic and
hemodynamic abnormalities
– Small LV cavity, septal hypertrophy which can be asymmetric
(ASH), systolic anterior motion of the mitral valve leaflet (SAM),
+/- obstruction of left ventricular outflow with low stroke
volume, but elevated EF

Source: UpToDate (www.uptodate.com) “Natural history of hypertrophic cardiomyopathy" Perry M Elliott, MD,
William J McKenna, MD, last updated 9/18/07

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Variants of HCM

8 - 10% 15 - 20%

65 -
70%

www.kanter.com/hcm
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 Dynamic LV outflow tract obstruction
 Outflow tract gradient (>30 mm Hg), considered severe if >50
mm Hg (occurs in 25-30% of cases leading to name hypertrophic
obstructive cardiomyopathy)
 Diastolic dysfunction
 Impaired diastolic filling,  filling pressure
 Myocardial ischemia
 Mitral regurgitation
 Arrhythmias

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Restrictive Cardiomyopathy

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Restrictive Cardiomyopathies

• Hallmark: abnormal diastolic function


• Rigid ventricular wall with impaired ventricular filling
• Bear/mmikul some functional resemblance to constrictive pericarditis
• Importance in its differentiation from operable constrictive pericarditis
• Much less common then DCM or HCM outside the tropics, but frequent cause of
death in Africa, India, South and Central America and Asia primarily because of
the high incidence of endomyocardial fibrosis in those regions

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Clinical Manifestations

• Symptoms of right and left heart failure


• Jugular Venous Pulse
• Echo-Doppler
– Abnormal mitral inflow pattern

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Treatment

• No satisfactory medical therapy


• Drug therapy must be used with caution
– Diuretics for extremely high filling pressures
– Vasodilators may decrease filling pressure
– (?) Calcium channel blockers to improve diastolic compliance
– Digitalis and other inotropic agents are not indicated

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