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Cardiomyopathy
Craig Ernst MHS, PA-C
Lock Haven University
Cardiomyopathy
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General term indicating disease of cardiac
muscle resulting in abnormal function
Divided into three types:
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Dilated cardiomyopathy-ventricular dilation
Hypertrophic cardiomyopathy-myocardial
hypertrophy
Restrictive cardiomyopathy-impaired
ventricular filling
Can have characteristics of more than one
Dilated Cardiomyopathy (DCM)
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Characterized by dilation and impaired
systolic function of left &/or right ventricle
Most common DCM is ischemic
cardiomyopathy
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Idiopathic (ICM) next most common
Familial autosomal dominant in 20% of cases.
Role of coxsackie/adenovirus and immune
mediated etiology unknown.
DCM
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Many cases of systemic heart muscle disease
present with features of DCM including:
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Ischemic/rheumatic CVD
Generalized disease- hemochromatosis, sarcoid
Connective tissue disease-SLE, systemic sclerosis
Neuromuscular disease-Friederich’s ataxia etc
Glycogen storage disease
Primary heart muscle disease- amyloidosis
Alcohol excess
Cytotoxic drugs-doxorubicin, cyclophosphamide
Pregnancy
Starling Curve
Contractility
Starling Curve
Volume
DCM
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Clinical features:
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R/L heart failure
Arrhythmia
Emboli
Cardiomegaly
Tachycardia
JVD
3rd/4th heart sounds
basiler crackles
displaced PMI
DCM
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Evaluation
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CXR: cardiomegaly
EKG: diffuse non specific ST-T wave changes,
LBBB common, tachycardia, conduction
abnormalities, arrhythmias
Echo: poor chamber contraction and dilated
chambers
If CAD suspected, cardiac catheterization
Endomyocardial biopsy for research only.
DCM
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Treatment
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Rx failure & arrhythmias
Ace Inhibitors a must in failure management
Non-specific Beta blockade:
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Carvedilol, ??, ??
Anticoagulation for A.fib/mural thrombus.
CRT-D (Bi-V AICD)
Transplant
Sudden death –
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Due to V. Tach. or V. Fib
Hypertrophic Cardiomyopathy (HCM)
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Complex heart disease due to the
asymmetric left ventricular hypertrophy,
left ventricular stiffness, mitral valve
changes and cellular changes (myocardial
disarray)
60 % inherited 40 % sporadic
HTN, Aging, Unknown
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Most autosomal dominant w/ variable
penetrance
Other Terms
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Hypertrophic Obstructive Cardiomyopathy
(HOCM)
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Idiopathic hypertrophic subaortic stenosis (IHSS)
Asymmetrical septal hypertrophy (ASH)
Systolic anterior motion (SAM) of mitral
apparatus
Pathophysiology
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HOCM is a subvalvular obstruction!
Distinct from valvular Aortic Stenosis
(pressure gradient across valve)
Gradient/obstruction increases with lower
LV volume
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HOCM pts here do better when full and slow
Standing after squatting/Valvsalva
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lower venous return & increase outflow
obstruction and intensity of murmur
MV leaflet
HCM without obstruction
HCM with obstruction HOCM)
HOCM: Clinical Features:
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Chest pain
Dyspnea
Syncope/Pre-syncope (typically with exertion)
Palpitations
Sudden Death (arrhythmia)
 Typically occurs in asymptomatic young adults or
adolescents (10-35 y/o)
 Family history of sudden death, sustained
ventricular tachycardia, & B/P response to exercise
are recognized risk factors
Diastolic dysfunction with impaired filling
Outflow tract obstruction occurs in 1/3 of cases
Physical Exam
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Systolic murmur; with little to no radiation to
neck vessels (increased by maneuvers that
decrease preload such as Valvsalva or
squatting)
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Decreases ventricular filling
May hear systolic murmur of MR
Weak late carotid pulse (late obstruction)
Diagnosed by echocardiogram.
Evaluation
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EKG-LVH with ST-T wave changes
CXR-normal
ECHO indicated if PE suggests IHSS
Pedigree analysis (ECHO to screen 1st
relatives)
Genetic analysis
XST/Holter
Treatment of HOCM
Relief of symptoms, prevention of endocarditis, arrhythmias and sudden death
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B-Blockers or verapamil
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Amiodarone or procainamide for A Fib
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need atrial kick to adequately fill LV
Avoid afterload reducing agents and vasodilators
(no ACEI/A2RB, NITRO)-refractory hypotension
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better filling, slow, bigger heart-less obstruction
These agents increase outflow obstruction
actually do better with increased SVR
Slow controlled emptying from increased SVR
SBE prophylaxis
Treatment of HOCM
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Implantable defibrillators
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may be indicated if at risk
for SCD
Dual-chamber pacemakers:
reverse of
resynchronization therapy
for LVEF
Surgical: myotomy &
myomectomy
Non surgical ablation of
the septum (alcohol
ablation through cath)
Sudden Cardiac Deaths
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2-3 % per year
Sudden & unexpected
Sudden death may be the initial (only) presentation
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Risk for SCD:
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NPR link
Extreme LVH
Family history of SCD
History of Vtach or syncope
Failure of BP to rise with exercise
Treatments that lower gradient do not prevent SCD
Restrictive Cardiomyopathy (RCM)
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May cause systolic & diastolic dysfunction
All increase LV stiffness
Characteristic ventricle filling pressures
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Over time filling dramatically ceases
Restrictive Cardiomyopathy
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Some cardiomyopathies do not present with dilation
or hypertrophy but rather restricted ventricular
filling (as with pericarditis)
Amyloidosis
Sarcoidosis
Hemochromoatosis
Endomyocardial fibrosis
Atrial dilation, atrial fibrillation and clot formation
common in restrictive
Restrictive Cardiomyopathy
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Clinical Features
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Dyspnea
Fatigue
Embolic phenomena
Elevated venous pressures
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JVD
Hepatomegaly
Edema
Ascites
Restrictive Cardiomyopathy
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CXR=cardiac enlargement
EKG-low voltage and ST-T wave
abnormalities (Exaggerated Septal Q’s – Think MI)
Echo-symmetrical myocardial thickening
Endomyocardial biopsy may be useful.
Is it restrictive pericarditis?
Pre-op effusion
Post op effusion
Restrictive Cardiomyopathy
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Treatment
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No specific treatment
Treat underlying cause… results?
Those with amyloidosis may recur after
transplant
Amyloidosis
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Systemic disorder, but if DHF occurs
usually from multiple myeloma
Fibrillar protein deposited throughout the
myocardium leading to rubbery consistency
and concentric hypertrophy
RV & LV hypertrophy
Absence of high voltage QRS on EKG
despite LVH on ECHO
Appearance on Echo
Amyloidosis
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Fat pad aspirate or tissue biopsies for
systemic amyloidosis
Endomyocardial biopsy if questionable
etiology
Poor prognosis
Hemochromatosis
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Hereditary disorder characterized by excess dietary iron
absorption and deposition in tissues with resulting endorgan damage.
Affects liver first and most frequently
Pancreatic involvement results in DM
Cardiac deposits leads to dilated cardiomyopathy
Skin deposits leads to bronze discoloration that results
from increased melanin production. Hyperpigmentation
Remember: liver, pancreas, heart
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Dx: AST, ALT, serum iron, TIBC, ferritin