心肌病完整版

CardiomyopathyandMyocarditisCardiomyopathiesDefinition “Aprimarydisorderoftheheartmusclethatcausesabnormalmyocardialperformanceandisnottheresultofdiseaseordysfunctionofothercardiacstructures…myocardialinfarction,systemichypertension,valvularstenosisorregurgitation”NewDefinitionCardiomyopathiesareaheterogeneousgroupofdiseasesofthemyocardiumassociatedwithmechanicaland/orelectricaldysfunctionthatusually(butnotinvariably)exhibitinappropriateventricularhypertrophyordilatationandareduetoavarietyofcausesthatfrequentlyaregenetic.Cardiomyopathieseitherareconfinedtotheheartorarepartofgeneralizedsystemicdisorders,oftenleadingtocardiovasculardeathorprogressiveheartfailure–relateddisability.Circulation2006;113;1807-1816WHOClassificationUnknowncause

(primary)DilatedHypertrophicRestrictiveARVCunclassifiedSpecificheartmuscledisease(secondary)IschemiccardiomyopathyValvularcardiomyopathyHypertensivecardiomyopathyInflammatorycardiomyopathyMetaboliccardiomyopathySystemicdiseaseMusculardystrophiesNeuromusculardisordersSensitivityandtoxicreactionsPeripartalcardiomyopathyCirculation93:841,1996

FunctionalClassificationDilatated(congestive,DCM,IDC)ventricularenlargementandsystdysfunctionHypertrophic(IHSS,HCM,HOCM)inappropriatemyocardialhypertrophy

intheabsenceofHTNoraorticstenosisRestrictive(infiltrative)abnormalfillinganddiastolicfunctionARVCUnclassifieddonotfitreadilyintoanycategory.Examplesincludesystolicdysfunctionwithminimaldilation,mitochondrialdisease,andfibroelastosis.ClassificationHypertrophicNormalDilatedHistopathologyofhypertrophicanddilatedcardiomyopathy.A,Thenormalarchitectureofhealthymyocardiumshowsorderlyalignmentofmyocyteswithminimalinterstitialfibrosis.B,Hypertrophiccardiomyopathy,demonstratingmarkedenlargementanddisarrayofmyocytes(red)withincreasedinterstitialfibrosis(blue).C,Dilatedcardiomyopathy,showinghypertrophyanddegenerationofmyocytes(darkred)withoutdisarray.Thereisanincreaseininterstitialfibrosis(palepink).Stains:AandC,hematoxylinandeosin;B,Massontrichrome.PrimaryCardiomyopathyCirculation2006;113;1807-1816Secondarycardiomyopathy1Circulation2006;113;1807-1816Secondarycardiomyopathy2Circulation2006;113;1807-1816IdiopathicDilatedCardiomyopathy14病例1：巨大左室的擴張性心肌病合并SMVTICD置入后，患者反復出現(xiàn)心悸、心累，自述心率120次/分。長期藥物治療。幾乎每月因室速住院一次。1+月前，再次出現(xiàn)心悸、伴大汗，四肢無力，血壓明顯下降入院治療。62歲男性，反復心悸、心累20+年，反復暈厥1年。2013年4月因心累、氣促，暈厥入院，診斷：擴張性心肌病、心功能III級、陣發(fā)性室性心動過速、心源性暈厥、甲亢（長期服用胺碘酮）。行ICD植入術(shù)。術(shù)前檢查心臟彩超：LV96mm，EF25%左房CT：左心室心腔明顯擴大，心包少量積液心電圖：陣發(fā)性室性心動過速，V1正向15術(shù)前檢查——胸部X片16院外體表心電圖——VT（起源于心外膜？）17術(shù)前體表心電圖——竇性心律18放置導管1910極CS導管20極Duo-Dec標測導管心包穿刺（LAO）20VT1激動傳導提示：外膜游離壁側(cè)起源21外膜游離壁處消融，VT1終止22IDC-DefinitionadiseaseofunknownetiologythatprincipallyaffectsthemyocardiumLVdilatationandsystolicdysfunctionpathologyincreasedheartsizeandweightventriculardilatation,normalwallthicknessheartdysfunctionoutofportiontofibrosisIncidenceandPrognosis3-10casesper100,00020,000newcasesperyearintheU.S.A.deathfromprogressivepumpfailure

1-year 25%

2-year 35-40%

5-year 40-80%stabilizationobservedin20-50%ofpatientcompleterecoveryisrareIdiopathicDilatedCardiomyopathy

ObservedSurvivalof104PatientsYearsAmJCardiol1981;47:525PredictingPrognosisinIDC

Predictive Possible NotPredictiveClinicalfactors symptoms alcoholism age

peripartum duration

familyhistory viralillnessHemodynamics LVEF LVsize

Cardiacindex atrialpressure Dysarrhythmia LVconddelay AVblock simpleVPC

complexVPC atrialfibrillation Histology myofibrilvolume otherfindingsNeuroendocrine hyponatremia

plasmanorepinephrine

atrialnatriureticfactorClinicalNoninvasiveInvasiveNYHAClassIII/IVLowLVejectionfractionHighLVfillingpressuresIncreasingageMarkedLVdilationLowexercisepeakoxygenconsumptionLowLVmassMarkedintraventricularconductiondelay≥ModeratemitralregurgitationComplexventriculararrhythmiasAbnormaldiastolicfunctionAbnormalsignal-averagedECGAbnormalcontractilereserveEvidenceofexcessivesympatheticstimulationRightventriculardilationordysfunctionProtodiastolicgallop(S3)

FactorsAssociatedwithanAdverseOutcomeinDilatedCardiomyopathy

HistoryandPhysicalExaminationSymptomsofheartfailurepulmonarycongestion(leftHF)

dyspnea(rest,exertional,nocturnal),orthpneasystemiccongestion(rightHF)

edema,nausea,abdominalpain,nocturialowcardiacoutput

fatigueandweaknesshypotension,tachycardia,tachypnea,JVDCardiacImagingChestradiogramElectrocardiogram24-hourambulatoryECG(Holter)lightheadedness,palpitation,syncopeTwo-dimensionalechocardiogramRadionuclideventriculographyCardiaccatheterizationage>40,ischemichistory,highriskprofile,abnormalECGChestX-rayConed-downviewofrightcostophrenicangleregionshowsthin,linearradiopaquelinesextendingtothepleuralsurface.TheseareKerleyBlines(thickenedinterlobularseptae)(arrows).Apicalfour-chamberviewofapatientwithadilatedcardiomyopathy.Notethedilationofallfourchambersandtherelativelysphericalgeometryoftheleftventricularcavity.EchocardiographyClinicalIndicationsforEndomyocardialBiopsyDefinitemonitoringofcardiacallograftrejectionmonitoringofanthracyclinecardiotoxicityPossibledetectionandmonitoringofmyocarditisdiagnosisofsecondarycardiomyopathiesdifferentiationbetweenrestrictiveandconstrictiveheartdiseaseManagementofDCMLimitactivitybasedonfunctionalstatussaltrestrictionofa2-gNa+(5gNaCl)dietfluidrestrictionforsignificantlowNa+initiatemedicaltherapyACEinhibitors,ARBdiureticsdigoxinhydralazine/nitratecombinationManagementofDCMconsideradding?-blockingagentsifsymptomspersistsanticoagulationforEF<30%,historyofthromboemoli,presenceofmuralthrombiintravenousdopamine,dobutamineand/orphosphodiesteraseinhibitorsBiventricularpacingBiventricularpacingThebiventricularpacingstrategyisbasedonthefactthatmostsubjectswithintraventricularconductiondelayhavedyssynchronousleftventricularcontraction,whichresultsinareductioninventricularperformanceandunfavorablemyocardialenergetics.Posteroanteriorandlateralchestx-raysdemonstratingtherightatrial,rightventricular(RV),andtheleftventricular(LV)leadsinapatientwithabiventricularpacemaker.TheLVlead(arrow)isplacedintheposterolateralveinbranchofthecoronarysinusallowingforsimultaneousactivationoftheseptumandtheLVfreewall.NormalsinusrhythmwithleftbundlebranchblockandQRSwidthof200ms.Atrial-sensedbiventricularstimulation;notehowthepacedQRScomplexhasnarrowedsignificantlyfromthebaseline.ManagementofDCMcardiactransplantationHypertrophicCardiomyopathy病例2EchoHypertrophicCardiomyopathyFirstdescribedbytheFrenchandGermansaround1900uncommonwithoccurrenceof2%to0.2%ahypertrophiedandnon-dilatedleftventricleintheabsenceofanotherdiseasesmallLVcavity,asymmetricalseptalhypertrophy(ASH),systolicanteriormotionofthemitralvalveleaflet(SAM)65%35%10%Schematicdiagramoftheleftventricleinhypertrophiccardiomyopathyduringsystole.Thereisprojectionofthebasalseptumintotheoutflowtractwithsystolicanteriormotionofthemitralvalve,whichresultsinleftventricularoutflowtractobstruction.Theobstructionisdynamic,dependentuponthepreload,afterload,andcontractilityoftheheart.EtiologyofHCMSchematicdiagramofthesarcomere.Hypertrophiccardiomyopathyisadiseaseofthesarcomere,withmutationsfoundofatleast10genescodingforvariouspartsofthesarcomere.PathophysiologySystoledynamicoutflowtractgradientDiastoleimpaireddiastolicfilling,fillingpressureMyocardialischemiamusclemass,fillingpressure,O2demand

vasodilatorreserve,capillarydensityabnormalintramuralcoronaryarteriessystoliccompressionofarteriesClinicalManifestationAsymptomatic,echocardiographicfindingSymptomaticdyspneain90%anginapectorisin75%fatigue,pre-syncope,syncope

riskofSCDinchildrenandadolescentspalpitation,CHF,dizzinesslessfrequentPhysicalExaminationFourthheartsound(S4)HyperdynamicapicalpulsePrecordialliftSystolicejectionmurmuratapexorlowerleftsternalborderMurmurincreasedwithvalsalvamaneuverIncreaseinGradientandMurmur

ContractilityPreloadAfterloavalsalva(strain) ---

standing ---

postextrasystole --

isoproterenol

digitalis

--amylnitrite --

nitroglycerine ---

exercise

tachycardia

--hypovolemia

DecreaseinGradientandMurmur

ContractilityPreloadAfterload

Muellermeneuver ---

valsalva(overshoot) ---

squatting ---

passivelegelevation --- phenylephrine ----- beta-blocker

--generalanesthesia

--

--isometricgrip -----

ECG1ECG2apicalvariantofhypertrophiccardiomyopathy

ChestX-RusuallynormalEchocardiogram(longaxis)Echocardiogram(shortaxis)EchocardiogramTwo-DimensionalEchocardiography

Massivehypertrophy

Asymmetricwallthickness

Sparklingorgranularappearanceofwalls

Normalcavitysize

Dilatedleftatrium

HyperdynamicLVfunction(EF>70)

Systolicanteriormotionofanterior(orposterior)mitralleaflet(obstructivecases)

Thickened,elongatedanteriorleaflet

EndocardiacthickeningofLVOT

HypodynamicbasalseptumDopplerEchocardiography

Mitralregurgitation

Mitralinflow:diastolicdysfunctionpatternwithimpairedrelaxationColor-FlowDoppler:HighvelocitiesandturbulentflowinLVOTOtherCausesofHypertrophyAorticstenosisClinicalmimicsglycogenstorage,infantsofdiabeticmothers,amyloidGeneticNoonan’s,Friedreich’sataxia,FamilialrestrictivecardiomyopathywithdisarrayExaggeratedphysiologicresponseAfro-Caribbeanhypertension,oldagehypertrophy,athlete’sheartHCMvsAthlete’sHeart

HCM Athlete + UnusualpatternofLVH -

+ LVcavity<45mm -

- LVcavity>55mm +

+ LAenlargement -

+ BizarreECGpaterns -

+ AbnormalLVfilling -

+ Femalegender -

- thicknesswithdeconditioning +

+ FamilyhistoryofHCM -Circulation1995;91:1596NaturalHistoryannualmortality3%inreferralcenters

probablycloserto1%forallpatientsriskofSCDhigherinchildren

maybeashighas6%peryear

majorityhaveprogressivehypertrophyclinicaldeteriorationusuallyisslowprogressiontoDCMoccursin10-15%Pathogenesisoftheend-stagephaseofhypertrophiccardiomyopathyRiskFactorsforSCDYoungage(<30years)“Malignant”familyhistoryofsuddendeathGenemutationspronetoSCD(ex.Arg403Gln)AbortedsuddencardiacdeathSustainedVTorSVTRecurrentsyncopeintheyoungNonsustainedVT(HolterMonitoring)Bradyarrhythmias(occultconductiondisease)BrHeartJ1994;72:S13RecommendationsforAthleticActivityAvoidmostcompetitivesports(whetherornotsymptomsand/oroutflowgradientarepresent)Low-riskolderpatients(>30yrs)mayparticipateinathleticactivityifallofthefollowingareabsentRecommendationsforAthleticActivityLow-riskolderpatients(>30yrs)mayparticipateinathleticactivityifallofthefollowingareabsentventriculartachycardiaonHoltermonitoringfamilyhistoryofsuddendeathduetoHCMhistoryofsyncopeorepisodeofimpairedconsciousnessseverehemdynamicabnormalities,gradient

50mmHgexerciseinducedhypotensionmoderateorsevermitralregurgitationenlargedleftatrium(

50mm)paroxysmalatrialfibrillationabnormalmyocardialperfusionManagementbeta-adrenergicblockerscalciumantagonistdisopyramideamiodarone,sotololDDDpacingICDAlcoholseptalAblationmyotomy-myectomyAlcoholseptalAblationAlgorithmRestrictiveCardiomyopathyRestrictiveCardiomyopathiesHallmark:abnormaldiastolicfunctionrigidventricularwallwithimpairedventricularfillingbearsomefunctionalresemblancetoconstrictivepericarditisimportanceliesinitsdifferentiationfromoperableconstrictivepericarditisExclusion“Guidelines”LVend-diastolicdimensions

7cmMyocardialwallthickness1.7cmLVend-diastolicvolume150mL/m2LVejectionfraction<20%ClassificationIdiopathicMyocardial 1.NoninfiltrativeIdiopathicScleroderma 2.InfiltrativeAmyloidSarcoidGaucherdiseaseHurlerdisease 3.StorageDiseaseHemochromatosisFabrydiseaseGlycogenstorageEndomyocardialendomyocardialfibrosisHyperesinophilicsyndCarcinoidmetastaticmalignanciesradiation,anthracyclineClinicalManifestationsSymptomsofrightandleftheartfailureJugularVenousPulseprominentxandydescentsEcho-DopplerabnormalmitralinflowpatternprominentEwave(rapiddiastolicfilling)reduceddecelerationtime(

LApressure)Constrictive-RestrictivePattern

“Square-RootSign”or“Dip-and-Plateau”RestrictionvsConstrictionHistoryprovidecanimportantcluesConstrictivepericarditishistoryofTB,trauma,pericarditis,sollagenvasculardisordersRestrictivecardiomyopathyamyloidosis,hemochromatosisMixedmediastinalradiation,cardiacsurgeryTreatmentNosatisfactorymedicaltherapyDrugtherapymustbeusedwithcautiondiureticsforextremelyhighfillingprssuresvasodilatorsmaydecreasefillingpressure?CalciumchannelblockerstoimprovediastoliccompliancedigitalisandotherinotropicagentsarenotindicatedArrhythmogenicRightVentricularCardiomyopathy/DysplasiaBackgroundPrevalanceestimatedat1:1000-5000,incidence6-44:10,000InnorthernItaly,accountsfor11%ofcasesofsuddencardiacdeathinyoungadults(numberonecauseafterhypertrophiccardiomyopathy)ReportsofhigherprevelanceinsouthernU.S.BackgroundMostcommonlypresentsbetweenages10and50years(mean30years)Higherincidenceinathleteshasbeenreported,suggestingcatecholaminemediatedmechanism3:1ratiomen:womenThirtypercentreportafamilyhistoryofeitherARVCorsuddencardiacdeathBackgroundFibrofattyreplacementofrightventricularmyocardiumleadingtodilatation,tachyarrhythmiasLeftventricleandseptumusuallysparedMostcommonlocationfortissuechangeisinthe“triangleofdysplasia”MallatZetal.NEnglJMed1996;335:1190-1197FreeWalloftheRightVentricleofaPatientwithRightVentricularDysplasiaPathophysiology“Diseaseofdesmosomaldysfunction”Desmosomesbindmyocardialcellstooneanother,providingcellularcontactnecessaryforelectricalconductionandmechanicalcontractionRightventricularwalland,inparticular,“triangleofdysplasia”,isthin,makingitpronetodisruptionExercisecausesincreasedchronotropyandinotropy.Thismayworsendisruptionandacceleratediseaseprocess.ClinicalManifestationsPalpitations:67%Syncope:32%Atypicalchestpain:27%Dyspnea:11%ClinicalManifestationsApproximately50%ofpatientswithARVCpresentwithventriculararrhythmiasMostcommonlysustainedornonsustainedventriculartachycardiathatoriginatesintheRV,thereforehasaLBBBpatternifcapturedonECG.VTcanbeexercisedinducedinpatientswithARVCDiagnosisDifferentialdiagnosisEvaluationDiagnosticcriteriaDifferentialDiagnosisUhl’sanomalyDeficiencyofRVmyocardiumAlmostequalmale:femalegenderratioPresentswithrightheartfailureDilatedcardiomyopathyinvolvingRVRareforARVCtohavemarkedandprogressiveLVdysfunctionLesslikelytohavesustainedVTorsuddencardiacdeathasinitialpresentationofDCMIdiopathicRVoutflowtracttachycardiaMorebenigncourse,canbetreatedwithradiofrequencyablationEvaluationECG40-50%havenormalECGonpresentationProlongedQRSComplete/incompleteRBBB30%havean“epsilonwave”,particularlyinV1(shownbestinleadsV1-V3onsignalaveragedECG)T-waveinversionsoverrightprecordialleadsEpsilonwaveεwaveEvaluationEchocardiographyRVdilatation,re