cardiomyopathy craig ernst mhs, pa-c lock haven university
TRANSCRIPT
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CardiomyopathyCraig Ernst MHS, PA-C
Lock Haven University
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Cardiomyopathy
General term indicating disease of cardiac muscle resulting in abnormal function
Divided into three types: Dilated cardiomyopathy-ventricular dilation Hypertrophic cardiomyopathy-myocardial
hypertrophy Restrictive cardiomyopathy-impaired
ventricular filling Can have characteristics of more than one
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Dilated Cardiomyopathy (DCM) Characterized by dilation and impaired
systolic function of left &/or right ventricle Most common DCM is ischemic
cardiomyopathy Idiopathic (ICM) next most common
Familial autosomal dominant in 20% of cases.
Role of coxsackie/adenovirus and immune mediated etiology unknown.
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DCM Many cases of systemic heart muscle disease
present with features of DCM including: 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
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Starling Curve
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Starling Curve
Volume
Con
tract
ility
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DCM Clinical features:
R/L heart failure Arrhythmia Emboli Cardiomegaly Tachycardia JVD 3rd/4th heart sounds basiler crackles displaced PMI
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DCM Evaluation
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.
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DCM Treatment
Rx failure & arrhythmias Ace Inhibitors a must in failure management Non-specific Beta blockade:
Carvedilol, ??, ??
Anticoagulation for A.fib/mural thrombus. CRT-D (Bi-V AICD) Transplant
Sudden death – Due to V. Tach. or V. Fib
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Hypertrophic Cardiomyopathy (HCM)
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
Most autosomal dominant w/ variable penetrance
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Other Terms Hypertrophic Obstructive Cardiomyopathy
(HOCM) Idiopathic hypertrophic subaortic stenosis (IHSS) Asymmetrical septal hypertrophy (ASH)
Systolic anterior motion (SAM) of mitral apparatus
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Pathophysiology
HOCM is a subvalvular obstruction! Distinct from valvular Aortic Stenosis
(pressure gradient across valve) Gradient/obstruction increases with lower
LV volume HOCM pts here do better when full and slow
Standing after squatting/Valvsalva lower venous return & increase outflow
obstruction and intensity of murmur
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HCM without obstruction
HCM with obstruction HOCM)
MV leaflet
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HOCM: Clinical Features: 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
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Physical Exam Systolic murmur; with little to no radiation to
neck vessels (increased by maneuvers that decrease preload such as Valvsalva or squatting) Decreases ventricular filling
May hear systolic murmur of MR Weak late carotid pulse (late obstruction) Diagnosed by echocardiogram.
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Evaluation 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
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Treatment of HOCM Relief of symptoms, prevention of endocarditis, arrhythmias and sudden death
B-Blockers or verapamil better filling, slow, bigger heart-less obstruction
Amiodarone or procainamide for A Fib need atrial kick to adequately fill LV
Avoid afterload reducing agents and vasodilators (no ACEI/A2RB, NITRO)-refractory hypotension These agents increase outflow obstruction actually do better with increased SVR Slow controlled emptying from increased SVR
SBE prophylaxis
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Treatment of HOCM Implantable defibrillators
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)
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Sudden Cardiac Deaths
2-3 % per year Sudden & unexpected Sudden death may be the initial (only) presentation
NPR link Risk for SCD:
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
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Restrictive Cardiomyopathy (RCM)
May cause systolic & diastolic dysfunction All increase LV stiffness Characteristic ventricle filling pressures
Over time filling dramatically ceases
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Restrictive Cardiomyopathy 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
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Restrictive Cardiomyopathy Clinical Features
Dyspnea Fatigue Embolic phenomena Elevated venous pressures
JVD Hepatomegaly Edema Ascites
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Restrictive Cardiomyopathy 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?
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Pre-op effusion Post op effusion
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Restrictive Cardiomyopathy Treatment
No specific treatment Treat underlying cause… results? Those with amyloidosis may recur after
transplant
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Amyloidosis 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
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Amyloidosis Fat pad aspirate or tissue biopsies for
systemic amyloidosis Endomyocardial biopsy if questionable
etiology
Poor prognosis
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Hemochromatosis Hereditary disorder characterized by excess dietary iron
absorption and deposition in tissues with resulting end-organ 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
Dx: AST, ALT, serum iron, TIBC, ferritin
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