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CARDIOMYOPATHY Athena Poppas, MD Associate Professor of Medicine, Brown Medical School Director,...
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CARDIOMYOPATHYCARDIOMYOPATHY
Athena Poppas, MDAthena Poppas, MD
Associate Professor of Medicine,Associate Professor of Medicine,
Brown Medical SchoolBrown Medical School
Director, Echocardiography Director, Echocardiography LaboratoryLaboratory
Rhode Island HospitalRhode Island Hospital
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CardiomyopathiesCardiomyopathies
Definition: diseases of heart muscleDefinition: diseases of heart muscle 1980 WHO: unknown causes1980 WHO: unknown causes
– Not clinically relevantNot clinically relevant 1995 WHO: “diseases of the 1995 WHO: “diseases of the
myocardium associated with myocardium associated with cardiac dysfunction “cardiac dysfunction “– pathophysiologypathophysiology– each with multiple etiologieseach with multiple etiologies
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CardiomyopathyCardiomyopathyWHO Classification
anatomy & physiology of the LV1. Dilated
• Enlarged • Systolic dysfunction
2. Hypertrophic• Thickened• Diastolic dysfunction
3. Restrictive• Diastolic dysfunction
4. Arrhythmogenic RV dysplasia• Fibrofatty replacement
5. Unclassified• Fibroelastosis• LV noncompaction
Circ 93:841, 1996
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CM: Specific EtiologiesCM: Specific Etiologies
IschemicIschemic ValvularValvular HypertensiveHypertensive InflammatoryInflammatory MetabolicMetabolic InheritedInherited Toxic reactionsToxic reactions PeripartumPeripartum
Ischemic: thinned, scarred tissue
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Dilated CardiomyopathyDilated Cardiomyopathy
•Dilation and impaired contraction of ventricles:•Reduced systolic function with or without heart failure•Characterized by myocyte damage •Multiple etiologies with similar resultant pathophysiology
•Majority of cases are idiopathic•incidence of idiopathic dilated CM 5-8/100,000•incidence likely higher due to mild, asymptomatic cases•3X more prevalent among males and African-Americans
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DCM: EtiologyDCM: EtiologyIschemicValvularHypertensiveFamilial Idiopathic Inflammatory
InfectiousViral – picornovirus, Cox B, CMV, HIVRicketsial - Lyme DiseaseParasitic - Chagas’ Disease, Toxoplasmosis
Non-infectiousCollagen Vascular Disease (SLE, RA)Peripartum
ToxicAlcohol, Anthracyclins (adriamycin), Cocaine
MetabolicEndocrine –thyroid dz, pheochromocytoma, DM, acromegaly,
NutritionalThiamine, selenium, carnitine
Neuromuscular (Duchene’s Muscular Dystrophy--x-linked)
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Prognosis depends on Prognosis depends on EtiologyEtiology
1230 pts. referred for unexplained CM. Felker GM. NEJM 2000;342:1077
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DCM: InfectiousDCM: Infectious
Acute viral myocarditisAcute viral myocarditis Coxasackie B or echovirusCoxasackie B or echovirus Self-limited infection in young Self-limited infection in young
peoplepeople Mechanism?:Mechanism?:
– Myocyte cell death and fibrosisMyocyte cell death and fibrosis– Immune mediated injuryImmune mediated injury– BUT:BUT:
No change with immunosuppressive drugsNo change with immunosuppressive drugs
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DCM: toxicDCM: toxic
Alcoholic cardiomyopathyAlcoholic cardiomyopathy Chronic useChronic use Reversible with abstinenceReversible with abstinence Mechanism?:Mechanism?:
– Myocyte cell death and fibrosisMyocyte cell death and fibrosis– Directly inhibits:Directly inhibits:
mitochondrial oxidative mitochondrial oxidative phosphorylationphosphorylation
Fatty acid oxidationFatty acid oxidation
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DCM: inheritedDCM: inherited
Familial cardiomyopathyFamilial cardiomyopathy 30% of ‘idiopathic’30% of ‘idiopathic’ Inheritance patternsInheritance patterns
– Autosommal dom/rec, x-linked, Autosommal dom/rec, x-linked, mitochondrialmitochondrial
Associated phenotypes:Associated phenotypes:– Skeletal muscle abn, neurologic, auditorySkeletal muscle abn, neurologic, auditory
Mechanism:Mechanism:– Abnormalities in:Abnormalities in:
Energy productionEnergy production Contractile force generationContractile force generation
– Specific genes coding for:Specific genes coding for: Myosin, actin, dystophin…Myosin, actin, dystophin…
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DCM: PeripartumDCM: PeripartumDiagnostic CriteriaDiagnostic Criteria 1 mo pre, 5 mos post1 mo pre, 5 mos post Echo: LV dysfunction Echo: LV dysfunction
– LVEF < 45% LVEF < 45% – LVEDD > 2.7 cm/m2 LVEDD > 2.7 cm/m2
Epidemiology/EtiologyEpidemiology/Etiology 1:4000 women1:4000 women
– JAMA 2000;283:1183JAMA 2000;283:1183
Proposed mechanisms: Proposed mechanisms: – Inflammatory Cytokines: Inflammatory Cytokines:
TNFa, IL6, Fas/AP01TNFa, IL6, Fas/AP01– JACC 2000 35(3):701.JACC 2000 35(3):701.
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PPCM: Prognosis PPCM: Prognosis Death from CM: ’91-97Death from CM: ’91-97
– 245 CM deaths in US, 0.88/100,000 live births, 245 CM deaths in US, 0.88/100,000 live births, 70% peripartum70% peripartum
– Increased risk with:Increased risk with: Maternal ageMaternal age AA 6.4x greaterAA 6.4x greater
– Whitehead SJ.Whitehead SJ. ObGyn2003;102:1326. ObGyn2003;102:1326.
Risk of recurrent pregnancyRisk of recurrent pregnancy– Retrospective survey : 44 women (16 vs 28)Retrospective survey : 44 women (16 vs 28)
Reduced EF, CHF 44% vs 21%, mortality 0 vs. 19%Reduced EF, CHF 44% vs 21%, mortality 0 vs. 19%– Elkyam U. NEJM.2001;344:1567.Elkyam U. NEJM.2001;344:1567.
– DSE:contractile reserve reduced in patients DSE:contractile reserve reduced in patients 7 women: change in Vcf7 women: change in Vcfc c σσESES relationship relationship
– Lampert MB. AJOG.1997.176.189.Lampert MB. AJOG.1997.176.189.
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Dilated Dilated CardiomyopathyCardiomyopathy
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MECHANISMS IN HEART FAILUREMECHANISMS IN HEART FAILURE
Hemodynamic DerangementClinical Heart FailureArrhythmia
NeurohormonesCytokinesOxidative stress
Ischemic injuryMyocardial diseaseGenetics
Altered molecular expressionAltered molecular expression
Ultrastructural changesUltrastructural changes
Myocyte hypertrophyMyocyte hypertrophy
Myocyte contractile Myocyte contractile dysfunctiondysfunction
ApoptosisApoptosis
Fibroblast proliferationFibroblast proliferation
Collagen depositionCollagen deposition
Ventricular remodelingVentricular remodeling
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PathophysiologyPathophysiology
•Initial Compensation for impaired myocyte contractility:•Frank-Starling mechanism•Neurohumoral activation intravascular volume
•Eventual decompensation•ventricular remodeling•myocyte death/apoptosis•valvular regurgitation
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Pathophysiology: Starling Pathophysiology: Starling CurveCurve
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Pathophysiology: Pathophysiology: NeurohumoralNeurohumoral
Adrenergic Adrenergic nervous systemnervous system
Renin-Renin-angiotensin-angiotensin-aldosterone axisaldosterone axis
VasopressinVasopressin Natriuretic Natriuretic
peptidespeptides EndothelinEndothelin
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Reduced Response to Adrenergic Reduced Response to Adrenergic StimulationStimulation
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Renin-Angiotensin-Aldosterone Renin-Angiotensin-Aldosterone PathwaysPathways
Angiotensinogen
Angiotensin-I
Angiotensin-II
Renin
ACE
AT-1 Receptor
Chymase
Bradykinindegradation
ACE-inhibitor
Angiotensinreceptorblocker
AldosteroneSpironolactone
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Angiotensin-II EffectsAngiotensin-II Effects
VasoconstrictionVasoconstriction Aldosterone Aldosterone
productionproduction Myocyte Myocyte
hypertrophyhypertrophy Fibroblast Fibroblast
proliferationproliferation Collagen depositionCollagen deposition
ApoptosisApoptosis Pro-thromboticPro-thrombotic Pro-oxidantPro-oxidant Adrenergic Adrenergic
stimulationstimulation Endothelial Endothelial
dysfunctiondysfunction
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The Kidney in Heart The Kidney in Heart FailureFailure
Reduced renal blood flowReduced renal blood flow Reduced glomerular filtration rateReduced glomerular filtration rate Increased renin production Increased renin production Increased tubular sodium Increased tubular sodium
reabsorptionreabsorption Increased free water retention Increased free water retention
(vasopressin)(vasopressin)
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Ventricular Ventricular Remodeling in Remodeling in Heart FailureHeart Failure
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Ventricular Remodeling following MI
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Extracellular Stimuli of Myocyte Extracellular Stimuli of Myocyte HypertrophyHypertrophy
Type Examples
Mechanical Stretch
Vasoactive peptides Angiotensin-IIEndothelin-1
adrenergic agonists Norepinephrine
Peptide growth factors Fibroblast GFInsulin-like GF
Cytokines TNF-
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Clinical FindingsClinical Findings
Biventricular Congestive Heart Failure
-Low forward Cardiac Output-fatigue, lightheadedness, hypotension
-Pulmonary Congestion-Dyspnea, -orthopnea, & PND
-Systemic Congestion-Edema-Ascites-Weight gain
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Physical ExamPhysical Exam
Decreased C.O.Tachycardia BP and pulse pressurecool extremities (vasoconstriction)Pulsus Alternans (end-stage)
Pulmonary venous congestion:ralespleural effusions
Cardiac:laterally displaced PMIS3 (acutely)mitral regurgitation murmur
Systemic congestion JVDhepatosplenomegalyascitesperipheral edema
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Diagnostic StudiesDiagnostic Studies
CXR -enlarged cardiac silhouette, vascular redistribution interstitial edema, pleural effusions
EKG –normaltachycardia, atrial and ventricular enlargement, LBBB, RBBB, Q-waves
Blood Tests (ANA,RF, Fe2+, TFT’s,ferritin,)
Echocardiography LV size, wall thickness functionvalve dz, pressures
Cardiac CatheterizationhemodynamicsLVEFangiography
Endomyocardial Biopsy
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Echo in dilated CMEcho in dilated CM
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Influence of EF on Survival in Influence of EF on Survival in Patients with Heart FailurePatients with Heart Failure
Vasan RS et al. J Am Coll Cardiol. 1999;33:1948-55
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Class 1: No limitation of physical activity. Class 1: No limitation of physical activity.
Ordinary physical activity w/o fatigue, palpitation, or dyspnea.Ordinary physical activity w/o fatigue, palpitation, or dyspnea.
Class 2: Slight limitation of physical activity. Comfortable at rest, but Class 2: Slight limitation of physical activity. Comfortable at rest, but symptoms w/ ordinary physical activitysymptoms w/ ordinary physical activity
Class 3: Marked limitation of physical activity. Comfortable at rest, but less Class 3: Marked limitation of physical activity. Comfortable at rest, but less than ordinary activity causes fatigue, palpitation, or dyspnea. than ordinary activity causes fatigue, palpitation, or dyspnea.
Class 4: Unable to carry out any physical activity without discomfort. Class 4: Unable to carry out any physical activity without discomfort. Symptoms include cardiac insufficiency at rest. If any physical Symptoms include cardiac insufficiency at rest. If any physical activity is undertaken, discomfort is increased. activity is undertaken, discomfort is increased.
J Cardiac Failure J Cardiac Failure 1999;5:357-3821999;5:357-382
Criteria for NYHA Functional Classification Criteria for NYHA Functional Classification
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Aim of TreatmentAim of Treatment
• Preload reduction• Diuretics• venodilators
• Vasodilators• ACEI
• Inotropes• Acutely• Chronically
• mortality
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Vasodilator Agents in Heart Vasodilator Agents in Heart FailureFailure
Drug Mechanism Action Use
Nitroglycerinand long-acting nitrates*
Direct via nitricoxide
Veno /arterioloar
Hemodynamic;anti-ischemic;long term
Nitroprusside Direct via nitricoxide
Arteriolar >venodilation
Hemodynamic
Hydralazine* Direct Arteriolar ?long term*
ACEinhibitors#
Reduced A-IIIncr. bradykinin
Veno /arterioloar
Long-term
*Hydralazine and a long-nitrate shown to reduce mortality long-term# Other actions (aside from vasodilation) likely to be important
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Dobutamine and Milrinone Dobutamine and Milrinone EffectsEffects
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Electrical and Electrical and Mechanical Ventricular Mechanical Ventricular
DyssynchronyDyssynchrony ExperimentallyExperimentally induced LBBB has effect on:induced LBBB has effect on:
– expressionexpression of regional stress kinasesof regional stress kinases– calcium-handling proteins.calcium-handling proteins.
Expression of p38-MAPKExpression of p38-MAPK (a stress kinase) is (a stress kinase) is elevated in the endocardiumelevated in the endocardium of the late-of the late-activated region, whereas phospholamban activated region, whereas phospholamban is decreased.is decreased.
Sarcoplasmatic reticulum CaSarcoplasmatic reticulum Ca2+2+-ATPase-ATPase is is decreased in the region of early activation.decreased in the region of early activation.
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Deleterious Deleterious Hemodynamic Effects of Hemodynamic Effects of LV DyssynchronyLV Dyssynchrony
Diminished SV & CO due:Diminished SV & CO due:
Reduced diastolic filling Reduced diastolic filling timetime11
Weakened contractility Weakened contractility 22
Protracted MV Protracted MV regurgitation regurgitation 22
Post systolic regional Post systolic regional contraction contraction 33
Atrio-ventricular
Inter-V
Intra-V
Cazeau, et al. PACE 2003; 26[Pt. II]: 137–143
1. Grines CL, Circulation 1989;79: 845-853 2. Xiao HB, Br Heart J 1991;66: 443-447 3. Søgaard P, JACC 2002;40:723–730
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CRT: Cardiac CRT: Cardiac Resynchronization Resynchronization
TherapyTherapy1. Improved 1. Improved
hemodynamicshemodynamics– Increased COIncreased CO– Reduced LV filling Reduced LV filling
pressurespressures– Reduced sympathetic Reduced sympathetic
activityactivity– Increased systolic Increased systolic
function w/o MVO2function w/o MVO22. Reverse LV 2. Reverse LV
remodeling/architectureremodeling/architecture– Decreased LVES/ED Decreased LVES/ED
volumesvolumes– Increased LVEFIncreased LVEF
– Circ ’02, JACC ’02, Circ ’02, JACC ’02, JACC ’02, NEJM’02JACC ’02, NEJM’02
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Risk of Sudden Death c/w Risk of Sudden Death c/w EFEF
Patients withoutPatients withoutLV DysfunctionLV Dysfunction
(LVEF >35%)(LVEF >35%)
Maggioni AP. GISSI-2 TrialGISSI-2 Trial Circulation. 1993;87:312-322.
Patients withLV Dysfunction
(LVEF < 35%)
No PVBs
1-10 PVBs/h
> 10 PVBs/h
0.86
A
0.88
0.90
0.92
0.94
0.96
0.98
1.00
0 30 60 90 120 150 180
Days
Su
rviv
al
p log-rank 0.002
0.88
0.90
0.92
0.94
0.96
0.98
1.00
0 30 60 90 120 150 180
DaysS
urv
ival
B
p log-rank 0.0001
0.86
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Anti-arrhythmic drugs, ICD Anti-arrhythmic drugs, ICD placebo and Deathplacebo and Death
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What are the two What are the two characteristic findings in characteristic findings in
DCM?DCM?
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Hypertensive Hypertensive Hypertrophic Hypertrophic
CardiomyopathyCardiomyopathy
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Women and HypertensionWomen and Hypertension
Prevalence of HTN in Women from NHANES-III. Burt VL. Hypertension ‘95
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Diastolic DysfunctionDiastolic Dysfunction
40-50% of pts w/ CHF 40-50% of pts w/ CHF have nml LVEFhave nml LVEF
– Vasan JACC ’99Vasan JACC ’99– Grossman Circ ‘00Grossman Circ ‘00
Prevalence:Prevalence:– increases with ageincreases with age– higher in womenhigher in women
Etiology: HTN & LVHEtiology: HTN & LVH Diagnosis:Diagnosis:
– MV& PV DopplerMV& PV Doppler– TDI, Color m-modeTDI, Color m-mode
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Zile MR. Circ;105:1387
Echo Doppler Echo Doppler ParametersParameters
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Diastolic DysfunctionDiastolic Dysfunction
Kawaguchi M. Circ 2003.107:714
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Zile MR. Circ;105:1387
Isolated Diastolic HFIsolated Diastolic HF
Isolated Systolic HFIsolated Systolic HF
Systolic & Diastolic HFSystolic & Diastolic HF
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What is the difference What is the difference between systolic and between systolic and diastolic LV dysfunction?diastolic LV dysfunction?
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Hypertrophic CardiomyopathyHypertrophic Cardiomyopathy
Left ventricular hypertrophy not due to pressure overload Hypertrpohy is variable in both severity and location:
-asymmetric septal hypertrophy-symmetric (non-obstructive)-apical hypertrophy
Vigorous systolic function, but impaired diastolic functionimpaired relaxation of ventricleselevated diastolic pressures
prevalence as high as 1/500 in general populationmortality in selected populations 4-6% (institutional)probably more favorable (1%)
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EtiologyEtiologyFamilial in ~ 55% of cases with autosomal dominant transmissionMutations in one of 4 genes encoding proteins of cardiac sarcomere
account for majority of familial cases
-MHCcardiac troponin T myosin binding protein C
-tropomyosinRemainder are spontaneous mutations.
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Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy
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Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy
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Hypertrophic cardiomyopathy
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Apical Hypertrophic
Cardiomopathy
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PathophysiologyPathophysiology
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HCM with outflow obstructionHCM with outflow obstructionDynamic LVOT obstruction (may not be present at rest)
SAM (systolic anterior motion of mitral valve)
LVOT Obstruction LVOT gradient wall stress MVO2 ischemia/angina
LVOT gradient: HR (DFP), preload (LVEDV), afterload(BP).
LVOT gradient: BP (Afterload), LVEDV(preload)
Symptoms of dyspnea and angina more related to diastolic dysfunction than to outflow tract obstructionSyncope: LVOT obstruction (failure to increase CO during exercise or after vasodilatory stress) or arrhythmia.
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Physical ExamPhysical Exam
Bisferiens pulse (“spike and dome”)S4 gallop Crescendo/Descrescendo systolic ejection murmur
HOCM vs. Valvular AS Intensity of murmurHOCMAS
Valsalva (preload, afterload) Squatting ( preload, afterload) Standing (preload, afterload)
Holosystolic apical blowing murmur of mitral regurgitation
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Diagnostic StudiesDiagnostic Studies
EKG– NSR– LVH– septal Q waves
2D-Echocardiography– LVH; septum >1.4x free wall– LVOT gradient by Doppler– Systolic anterior motion of
the mitral valeregurgitation Cardiac Catheterization
– LVOT gradient and pullback– provocative maneuvers– Brockenbrough phen
HCM-ASH using contrast
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Cardiac CatheterizationCardiac Catheterization
LV pullback
Brockenbrough-Braunwald Signfailure of aortic pulse pressure to rise post PVC
Provocative maneuvers:Valsalvaamyl nitrate inhalation
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Atrial FibrillationAtrial FibrillationAcute A. Fib is poorly tolerated -Acute Pulmonary Edema and ShockChronic a fib - Fatigue, dyspnea and angina
Rapid HR - decreased time for diastolic filling and LV relaxationLoss of atrial “Kick” – decreased LV filling
- decreased SV and increased outflow tract obstruction
Rate slowing with -blockers and Ca2+ channel blockers Digitalis is relatively contra-indicated- positive inotropeDC Cardioversion
No p wave P wave present
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TreatmentTreatmentFor symptomatic benefit-blockers
mvO2 gradient (exercise)arrythmias
Calcium Channel blockersAnti-arrhythmics
afibamiodoroneDisopyramide
AICD for sudden death
antibiotic prophylaxis for endocarditisNo therapy has been shown to improve mortality
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HCM: Surgical TreatmentHCM: Surgical Treatment
For severe symptoms with large outflow gradient (>50mmHg)Does not prevent Sudden Cardiac Death
Myomyectomyremoval of small portion of upper IV septum +/- mitral valve replacement
5 year symptomatic benefit in ~ 70% of patients
Dual Camber (DDD pacemaker) pacingdecreases LVOT gradient (by~25%)randomized trials have shown little longterm benefitpossible favorable morphologic changes
ETOH septal ablation
AICD to prevent sudden death
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Hypertrophic CMHypertrophic CM Most common cause of death in young people. Most common cause of death in young people.
The magnitude of left ventricular hypertrophy is The magnitude of left ventricular hypertrophy is directly correlated to the risk of SCD. directly correlated to the risk of SCD.
Young pts with extreme hypertrophy and few or no Young pts with extreme hypertrophy and few or no symptoms are at substantial long-term risk of SCD.symptoms are at substantial long-term risk of SCD.
.Spirito P. N Engl J Med. 1997;336:775-785. Maron BJ. N Engl J Med. 2000;342:365-373.
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Wall Thickness and Wall Thickness and Sudden Death in HCMSudden Death in HCM
Spirito P. N Engl J Med. 2000;342:1778-1785.
02468
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< 15 16-19 20-24 25-29 > 30
Maximum Left-Ventricular-Wall Thickness (mm)
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18.2
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PrognosisPrognosisSudden Death 2-4%/year in adults
4-6% in children/adolescentsAICD for: survivors of SCD with Vfib
episodes of Sustained VTpts with family hx of SCD in young family membersHigh risk mutation (TnT, Arg403Gln)
Predictors of adverse prognosis:early age of diagnosisfamilial form with SCD in 1st degree relativehistory of syncopeischemiapresence of ventricular arrhythmias on Holter (EPS)
EPSAmiodorone (low dose)Prophylactic AICD?
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HCM vs Athletes HeartHCM vs Athletes Heart
Endurance training:Endurance training:– Physiologic increase in LV massPhysiologic increase in LV mass
Wall thickness and cavity sizeWall thickness and cavity size
Early HCM vs Athlete’s heartEarly HCM vs Athlete’s heart– DEFINITION: Symmetric, <13mmDEFINITION: Symmetric, <13mm– 947 elite athletes: 16 thickness=13-16mm947 elite athletes: 16 thickness=13-16mm
15 rowers, EDD=55-63 c/w 728 athletes/22 other15 rowers, EDD=55-63 c/w 728 athletes/22 other NEJM1991;324:295NEJM1991;324:295
– 286 cyclists: 25 thickness 13-15286 cyclists: 25 thickness 13-15 50% increased EDD w/ 12% reduced LVEF50% increased EDD w/ 12% reduced LVEF
JACC 2004;44:144.JACC 2004;44:144.
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Why do patients with Why do patients with HCM develop heart HCM develop heart failure?failure?
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Restrictive Restrictive CardiomyopathyCardiomyopathy
Characterized by:• impaired ventricular filling due to an abnormally stiff (rigid) ventricle•normal systolic function (early on in disease)•intraventricular pressure rises precipitously with small increases in volume
Pressure
Volume
Causes : infiltration of myocardium by abnormal substancefibrosis or scarring of endocardium
normal
restriction
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Amyloid infiltrative CM
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AmyloidosisAmyloidosis
Primary Amyloidosisimmunoglobulin light chains -- multiple myeloma
Secondary Amyloidosisdeposition of protein other than immunoglobulin
senilefamilialchronic inflammatory process
restriction caused by replacement of normal myocardial contractile elements by infiltrative interstitial deposits
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AmyloidosisAmyloidosis
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Amyloid Amyloid CardiomyopathyCardiomyopathy
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SarcoidosisSarcoidosis
RestrictionConduction System DiseaseVentricular Arrhythmias (Sudden Cardiac Death)
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Endomyocardial FibrosisEndomyocardial Fibrosis
Endemic in parts of Africa, India, South and Central America, Asia15-25% of cardiac deaths in equatorial Africa
hypereosinophilic syndrome (Loffler’s endocarditis)
Thickening of basal inferior wallendocardial deposition of thrombusapical obliterationmitral regurgitation80-90% die within 1-2 years
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Pathophysiology of Pathophysiology of RestrictionRestriction
Elevated systemic and pulmonary venous pressuresright and left sided congestion
reduced ventricular cavity size with SV and CO
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Clinical FindingsClinical Findings
Right > Left heart failureDyspneaOrthopnea/PNDPeripheral edemaAscites/Hepatomegaly
Fatigue/ exercise tolerance
Clinically mimics constrictive Pericarditis
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Diagnostic StudiesDiagnostic Studies
2D-Echo/Doppler-mitral in-flow velocityrapid early diastolic filling
Catheterization – diastolic pressure equilibration restrictive vs constrictive
hemodynamics
Endomyocardial biopsy- definite Dx of restrictive pathology
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Cardiac CatheterizationCardiac Catheterization
Prominent y descent “dip and plateau” rapid atrial emptying rapid ventricular fillingthen abrupt cessation of blood flow due to non-compliant myocardium
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Constriction vs. Restrictive CMConstriction vs. Restrictive CM
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TreatmentTreatmentTreat underlying cause
r/o constriction which is treatable (restriction poor prognosis)amyloid (melphalan/prednisone/colchicine)Endomyocardial Fibrosis (steroids, cytotoxic drugs, MVR)Hemochromatosis (chelation, phlebotomy)
Sarcoidosis (steroids)
DiureticsFor congestive symptoms, but LV/RV filling CO
Digoxin (avoid in amyloidosis)Antiarrhythmics for afib
amiodoronePacemaker for conduction system diseaseAnticoagulation for thrombus (esp in atrial appendages)
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What is the What is the hemodynamic problem hemodynamic problem in RCM?in RCM?
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Arrhythmogenic RV Arrhythmogenic RV DysplasiaDysplasia
Myocardium of RV free wall Myocardium of RV free wall replaced:replaced:– Fibrofatty tissue Fibrofatty tissue – Regional wall motion/function is Regional wall motion/function is
reducedreduced Ventricular arrhythmiasVentricular arrhythmias
– SCD in youngSCD in young
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MRI: RV DysplasiaMRI: RV Dysplasia
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LV NoncompactionLV Noncompaction
Diagnostic CriteriaDiagnostic Criteria Prominent trabeculations, deep recesses in
LV apex Thin compact epicardium, thickened
endocardium Stollberger C, JASE ‘04
Other phenotypic findingsPrognosis and TreatmentPrognosis and Treatment Increased risk of CHF, VT/SCD, thrombosis
Oechslin EN, JACC ‘00 Hereditary risk
– Screening of offspring Pregnancy: case reportPregnancy: case report
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Echo: LV NoncompactionEcho: LV Noncompaction
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CardiomyopathyCardiomyopathyWHO Classification
anatomy & physiology of the LV1. Dilated
• Enlarged • Systolic dysfunction
2. Hypertrophic• Thickened• Diastolic dysfunction
3. Restrictive• Myocardial stiffness• Diastolic dysfunction
4. Arrhythmogenic RV dysplasia• Fibrofatty replacement
5. Unclassified• Fibroelastosis• LV noncompaction