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

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

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

CM: Specific EtiologiesCM: Specific Etiologies

IschemicIschemic ValvularValvular HypertensiveHypertensive InflammatoryInflammatory MetabolicMetabolic InheritedInherited Toxic reactionsToxic reactions PeripartumPeripartum

Ischemic: thinned, scarred tissue

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

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)

Prognosis depends on Prognosis depends on EtiologyEtiology

1230 pts. referred for unexplained CM. Felker GM. NEJM 2000;342:1077

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

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

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…

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.

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.

Dilated Dilated CardiomyopathyCardiomyopathy

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

PathophysiologyPathophysiology

•Initial Compensation for impaired myocyte contractility:•Frank-Starling mechanism•Neurohumoral activation intravascular volume

•Eventual decompensation•ventricular remodeling•myocyte death/apoptosis•valvular regurgitation

Pathophysiology: Starling Pathophysiology: Starling CurveCurve

Pathophysiology: Pathophysiology: NeurohumoralNeurohumoral

Adrenergic Adrenergic nervous systemnervous system

Renin-Renin-angiotensin-angiotensin-aldosterone axisaldosterone axis

VasopressinVasopressin Natriuretic Natriuretic

peptidespeptides EndothelinEndothelin

Reduced Response to Adrenergic Reduced Response to Adrenergic StimulationStimulation

Renin-Angiotensin-Aldosterone Renin-Angiotensin-Aldosterone PathwaysPathways

Angiotensinogen

Angiotensin-I

Angiotensin-II

Renin

ACE

AT-1 Receptor

Chymase

Bradykinindegradation

ACE-inhibitor

Angiotensinreceptorblocker

AldosteroneSpironolactone

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

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)

Ventricular Ventricular Remodeling in Remodeling in Heart FailureHeart Failure

Ventricular Remodeling following MI

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-

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

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

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

Echo in dilated CMEcho in dilated CM

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

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

Aim of TreatmentAim of Treatment

• Preload reduction• Diuretics• venodilators

• Vasodilators• ACEI

• Inotropes• Acutely• Chronically

• mortality

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

Dobutamine and Milrinone Dobutamine and Milrinone EffectsEffects

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.

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

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

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

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0.88

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Anti-arrhythmic drugs, ICD Anti-arrhythmic drugs, ICD placebo and Deathplacebo and Death

What are the two What are the two characteristic findings in characteristic findings in

DCM?DCM?

Hypertensive Hypertensive Hypertrophic Hypertrophic

CardiomyopathyCardiomyopathy

Women and HypertensionWomen and Hypertension

Prevalence of HTN in Women from NHANES-III. Burt VL. Hypertension ‘95

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

Zile MR. Circ;105:1387

Echo Doppler Echo Doppler ParametersParameters

Diastolic DysfunctionDiastolic Dysfunction

Kawaguchi M. Circ 2003.107:714

Zile MR. Circ;105:1387

Isolated Diastolic HFIsolated Diastolic HF

Isolated Systolic HFIsolated Systolic HF

Systolic & Diastolic HFSystolic & Diastolic HF

What is the difference What is the difference between systolic and between systolic and diastolic LV dysfunction?diastolic LV dysfunction?

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%)

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.

Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy

Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy

Hypertrophic cardiomyopathy

Apical Hypertrophic

Cardiomopathy

PathophysiologyPathophysiology

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.

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

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

Cardiac CatheterizationCardiac Catheterization

LV pullback

Brockenbrough-Braunwald Signfailure of aortic pulse pressure to rise post PVC

Provocative maneuvers:Valsalvaamyl nitrate inhalation

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

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

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

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.

Wall Thickness and Wall Thickness and Sudden Death in HCMSudden Death in HCM

Spirito P. N Engl J Med. 2000;342:1778-1785.

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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?

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.

Why do patients with Why do patients with HCM develop heart HCM develop heart failure?failure?

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

Amyloid infiltrative CM

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

AmyloidosisAmyloidosis

Amyloid Amyloid CardiomyopathyCardiomyopathy

SarcoidosisSarcoidosis

RestrictionConduction System DiseaseVentricular Arrhythmias (Sudden Cardiac Death)

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

Pathophysiology of Pathophysiology of RestrictionRestriction

Elevated systemic and pulmonary venous pressuresright and left sided congestion

reduced ventricular cavity size with SV and CO

Clinical FindingsClinical Findings

Right > Left heart failureDyspneaOrthopnea/PNDPeripheral edemaAscites/Hepatomegaly

Fatigue/ exercise tolerance

Clinically mimics constrictive Pericarditis

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

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

Constriction vs. Restrictive CMConstriction vs. Restrictive CM

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)

What is the What is the hemodynamic problem hemodynamic problem in RCM?in RCM?

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

MRI: RV DysplasiaMRI: RV Dysplasia

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

Echo: LV NoncompactionEcho: LV Noncompaction

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