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7/30/2019 MF3 - Cardiomyopathy
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CARDIOMYOPATHY
April Grace A. Chiongbian
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Group of diseases that primarily affect the
heart muscle.
Not a result of congenital, acquired, valvular,
hypertensive, coronary arterial or pericardial
abnormalities
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Three Morphologic Types
Dilated Cardiomyopathy
Restrictive Cardiomyopathy
Hypertrophic Cardiomyopathy
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EPIDEMILOGY
Endomyocardial Fibrosis
Children and young adults
Tropical and subtropical Africa
Frequent cause of CHF in Africa
Dilated Cardiomyopathy in Africa
Infection with Toxoplasma gondii and Coxsackievirus B
(Nigeria)
African Trypanosomiasis (Cameroon)
of patient hospitalized with CHF
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Peripartum DCM
More common in Africa than in North Aerica and
Europe
Low socio-economic Status
High Parity
Excessive Salt intake
Selenium deficiency
Prolonged lactation
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Commonly presents in 3rd to 4th decade of life
Cardiomyopathy in Asia
Thiamine Deficiency
Wet beri-beri heart disease
Selenium Deficiency
Keshans Disease\
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DILATED CARDIOMYOPATHY
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Ventricular chamber enlargement
Systolic pump function is impaired leading toprogressive cardiac dilatation
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Etiology
Familial
End result of myocardial damage due to
known or unknown Infectious, metabolic or
toxic agents
Late consequence of acute viral myocarditis
Reversible form may be found with alcohol
abuse, pregnancy, thyroid disease, cocaine use
and chronic uncontrolled tachycardia
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Alcoholic Cardiomyopathy
Peripartum Cardiomyopathy
Neuromuscular Diseases Drugs
Arrhythmogenic Right Ventricular
Cardiomyopathy/ Dysplasia (ARVC/D)
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Clinical Features
Symptoms develop gradually
LV dilatation occurs for months or years before
becoming symptomatic
Vague chest pain
Syncope due to arrythmias and systemic
embolism
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Physical Examination
Narrow pulse pressue
Jugular venous pressure is elevated
Third and fourth heart sounds Mitral or tricuspid regurgitation
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Laboratory Examination
Chest Roegenogram enlargement of cardiac
sillhouette
ECG left bundle branch block and ST-T wave
abnormality
CTI and CMRI LV dilatation
Cardiac Catheterization
Coronary angiography dilated, diffusely
hypokinetic left ventricle
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ALCOHOLIC CARDIOMYOPATHY
>90g/d of alcohol
Risk of developing cardiomyopathy is partially
determined genetically
Polymorphism of the gene encoding alcohol
dehydrogenase as well as the DD form of ACE
gene
Poor prognosis (
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Holiday Heart Syndrome Second presentation of alcoholic cardiotoxicity
Recurrent supraventricular or ventricular
tachyarrhythmias Appears after a drinking binge
Atrial fibrillation
Atrial flutter
Frequent ventricular premature depolarizations
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PERIPARTUM CARDIOMYOPATHY
Last trimester of pregnancy or within 6
months of delivery
Cause: Inflammatory myocarditis, immune
activation and gestational hypertension
Multiparous, African ancestry, >30 years old
Prognosis:
Poor: LV remains enlarged and/ or LV ejection
fraction remains depressed after 6 months
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NEUROMUSCULAR DISEASE
In Duchennes progressive muscular
dystrophy, mutations in genes that encode
dystrophin lead to mycocyte death
Tall R waves in right precordial leads with an
R/S ration >1.0, often associated with deep Q
waves in limb and lateral precordial leads
Supraventricular and ventricular arrhythmias
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Myocardial Dystrophy
Disorders of impulse formationn and AV
conduction
Syncope ad sudden death are major haards
Insertion of an ICD and/or permanent pacemaker
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DRUGS
Anthracycline derivatives
Systolic dysfunction and ventricular arrhythmias
Cardiotoxicity 3 months after last dose
Late contractile dysfunction Prevention: document preclinical deterioration of LV
function
Modification of dose and dose schedule
Take along with cardioprotective agents (iron-chelatordexrazoxone)
ACE inhibitors
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Trastuzumab
Cyclophosphamide
Imatinib mesylate Cocaine abuse
Cardiac complications: SCD, Myocarditis, DCM and
acute MI
Nitrates, calcium channel blockers, antiplatelet
agents and benzodiazepines
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Arrhythmogenic Right Ventricular
Cardiomyopathy/ Dysplasia (ARVC/D)
Familial
Progressive fibrofatty replacement of the right
ventricle and LV myocardium
Autosomal dominant
Mutations in desmosomes
Plakophilin-2 most common
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RV failure with Jugular venous distention
Hepatomegaly
Edema
Manifest during 2nd decade Ventricular tachyarrhythmias
RV failure
ECG
QRS prolongation
CT-Scan/ MRI
RV dilatation, RV aneurysm ad Fatty replacement
MANAGEMENT:
Restriction from Competitive sports
Antiarrhythmic therapy with beta blockers or amiodarone
TAKO TSUBO (stress)
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TAKO-TSUBO (stress)
CARDIOMYOPATHY
Apical Ballooning syndrome Abrupt onset of severe chest discomfort
Preceded by very stressful emotional or physical
event
Women >50 y/o
ST segment elevations; Deep T wave inversions
Severe akinesia of LV
Troponins mildly elevated
Reversible within 3-7 days
Adrenergic Surge involved mechanism
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LEFT VENTRICULAR NONCOMPACTION
Uncommon congenital
Present at any age (CHF, thromboembolism,
Ventricular arrhythmias)
Arrest of normal embryogeneis
Echocariography
Multple deep trabeculations into the myocardium
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TREATMENT
Standard therapy for Heart Failure
Cardiac Resynchronization Therapy
Insertion of Implantalbe CardioverterDefibrillator
Cardiac Transplantation
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HYPERTROPHIC
CARDIOMYOPATHY
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LV hypertrophy
Two Features:
Asymmetric LV hypertrophy
Dynamic LV outflow tract pressure gradient
Diastolic Dysfunction ubiquitous
pathophysiologic abnormality Distinctive pattern of hypertrophy
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CLINICAL FEATURES
First Clinical Manifestation: Sudden Cardiac
Death
Dyspnea most common complaint
Syncope
Angina pectoris
Fatigue
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PHYSICAL EXAMINATION
Double or triple apical precordial impulse
Fourth heart sound
Systolic Murmur
Hallmark of obstructive HCM
Typically harsh, diamond-shaped and begins well
after the first heart sound.
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HEMODYNAMICS
Pressure gradient is dynamic
Obstruction is due to narrowing of LV outflow
tract by systolic anterior movement of the
mitral valve against the hypertrophied septum
Three basic mechanisms:
Increased LV contractility
Decreased ventricular preload
Decreased aortic impedance and pressure
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LABORATORY EXAMINATIONS
ECG: LV hypertrophy and widespread, deep
broad Q waves.
Echocardiogram
Mainstay of diagnosis
LV hypertrophy, septum
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PROGNOSIS
SCD is major cause of mortality
Atrial Fibrillation common late in the disease
Infective Endocarditis
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INHERITED METABOLIC CM with LV
HYPERTROPHY
Cardiac Danon Disease
Glycogen Storage Cardiomyopathy
Fabry Disease
Friedreichs Ataxia
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TREATMENT
B- adrenergic blockers
Amiodarone
Nindihydropyridine calcium channel blockers
(verapmil and diltiazem)
Surgical Myotomomy/ Myectomy
Insertion of ICD
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RESTRICTIVE CARDIOMYOPATHY
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Abnormal diastolic function
Ventricular walls are excessively rigid and
impede ventricular filling
Partial obliteration of the ventricular cavity by
fibrous tissue and thrombus contributes to the
abnormally increased resistance
CLINICAL FEATURES
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CLINICAL FEATURES
Exercise intolerance
Dyspnea
Dependent edema
Ascites
Enlarged tender and often pulsatile liver
Elevated jugular venous pressure and does not
fall normally with inspiration (Kussmauls sign)
3rd and 4th heart sounds
Apex impulse easily palpable
Mitral regurgitation is common
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LABORATORY EXAMINATION
ECG:Low voltage, nonspecific ST-T wave abnormalitiesand various arrhythmias
Echocardiography, CTI and CMRI: symmetrically
thickened LV walls and normal or slightly reducedventricular volumes and systolic function; Atria usuallydilated
Doppler:Diastolic Dysfunction
Cardiac Catherterization: reduced cardiac output,elevation of LV and RV end-diastolic pressures, and a dip-and-plateau configuration
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ETIOLOGIES
Eosinophilic Endomyocardial Disease
Cardiac Amyloidosis
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EOSINOPHILIC ENDOMYOCARDIAL
DISEASE
Loefflers endocarditis or Fibroplastic Endocarditis
Occurs in temperate climates
Endocardium of either or both ventriles is thickened
Mitral regurgitation present on Doppler Large mural thrombi may develop in either ventricle
Hepatosplenomegaly and localized infiltration of otherorgans are present
Management: Diuretics, afterload-reducing agnets andanticoagulation
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CARDIAC AMYLOIDOSIS
Four clinical presentations:
Diastolic dysfunction
Systolic dysfunction
Arrhythmias and conduction disturbances
Orthostatic hypotension
2d-Echo: show thickened myocardial wall with a
diffuse, hyperrefractile speckled appearance Management: Chemotherapy, Heart
transplantation
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Other RCM
Iron-overload cardiomyopathy
(hemochromatosis)
Myocardial Sarcoidosis
Carcinoid Syndrome
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TREATMENT
Salt restriction
Diuretics
Digitalis impaired systolic function
Anticoagulation eosinophilic
endomyocarditis
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OT Management
Energy Conservation Techniques
Relaxation Techniques
Patient Education and Supportive Therapy