內科學教學課件：Cardiomyopathy

1Cardiomyopathy2DefinitionDiseaseofthemyocardiumassociatedwithcardiacdysfunction.nottheresultofdiseaseordysfunctionofothercardiacstructures…myocardialinfarction,systemichypertension,valvularstenosisorregurgitation”3Classification(WHO1995)

acceptedworld-wideprimaryDilatedCardiomyopathy(DCM)HypertrophicCardiomyopathy(HCM)RestrictiveCardiomyopathy(RCM)ArrhythmogenicrightventricularCardiomyopathy(ARVC)UnclassifiedCardiomyopathySpecificDilatedCardiomyopathy,DCMDilatedcardiomyopathyisaprogressivediseaseofheartmusclethatischaracterizedbyventricularchamberenlargementandcontractiledysfunctionwithnormalleftventricular(LV)wallthickness.Therightventriclemayalsobedilatedanddysfunctional.

5EtiologyInheriteddiseaseInfections,viral…ToxinsNutritionalInfiltrativeMetabolic

……

geneticmutations

thecurrent5(LMNA,MYH7,TNNT2,SCN5A,MYBPC3)novelmutations(TTN,BAG3)infamilialdilatedcardiomyopathy.Findingaspecificcauseforanindividualcasemaybedifficult.6EpidemiologyThetrueincidenceofcardiomyopathiesisunknown.

19casesper100,000inChina,recentlyreported.

3-10casesper100,000;20,000newcasesperyearintheU.S.A.7PathologyIncreasedheartsizeandweightVentriculardilatation,normalwallthicknessFibrosisThrombosis8Fig1DilatedCardiomyopathyDilatedheart,wallthickness9Fig2DilatedCardiomyopathy

Myocardialfibrosis10Fig3DilatedCardiomyopathy

Thrombosis

11PathophysiologyEarlycompensatorymechanismsleadtofurthermyocardialinjury,dysfunction,andgeometricremodelingNeurohormonalactivationCirculatingcytokinesasmediatorsofmyocardialinjury12ClinicalmanifestationOccursinanyage,esp.in30－50years

old.Men2timesmorefrequentthanwomen.Theincidenceisabout4-8per100000person-yearsandtheprevalence36.5per100000individuals.Symptomsmaybegradual.13IdiopathicDilatedCardiomyopathy

ObservedSurvivalof104PatientsYearsAmJCardiol1981;47:52514Symptomsheartfailurepulmonarycongestion(leftHF)

dyspnea(exertional,atrest,paroxysmalnocturnaldyspnea,orthopnea)systemiccongestion(rightHF)

edema,nausea,abdominalpain,nocturialowcardiacoutput

fatigueandweaknessArrhythmias(VPB,VT,AF)Cardiacsuddendeath15SignscardiacdilatationThe3rd,4thheartsoundsGalloprhythmMitralandtricuspidregurgitationmurmursEmbolization16AuxiliaryexaminationEchocardiography

bigchambersizes,normalwallthickness,relativelysmallervalvularcavity,loweramplitudeoftheheartpulsateandthrombosis.Chestradiographs

cardiomegaly,KerleyBlines,alveolaredema.ElectrocardiographyNonspecificCatheterizationEndomyocardialbiopsy17Two-dimensionalechocardiography

inDCMTheheartchambersaredilated.18M-modelechocardiographyinDCMEPSS19DopplerechocardiographyinDCMRegurgitationofthemitralandtricuspidvalve.20Two-dimensionalechocardiographyinDCMEnlargedheart.LateralthrombusisshownattheLVapex.21Fig4theenlargedheart22DiagnosisThesymptomsandsignsofsystolicdysfunction.Thetypicalpresentationofechocardiogram.Ruleoutothercardiacdiseases.23Essentiallythesameastreatmentofchronicheartfailure(CHF).

Drugclassesusedincludethefollowing:TreatmentAngiotensin-convertingenzymeinhibitors(ACEI)AngiotensinIIreceptorblockers(ARB)Beta-blockersAldosteroneantagonistsCardiacglycosidesDiureticsVasodilators

24TreatmentcontinuedAnticoagulants

Warfarin

Patientsinatrialfibrillation,withknownmuralthrombus,withembolismhistoryorwithlowerejectionfractionvalues(<30%).

Antiarrhythmics

AmiodaroneDofetilideImplantablecardioverterdefibrilator(ICD)25Treatmentcontinued

Cardiacresynchronizationtherapy(CRT)Biventricularpacingwithleadspositioned

intherightatrium,rightventricle,andcoronarysinus.

Thebenefitswereconfirmedinmultiplestudiesfromthemid-1990s.26Fig5BiventricularPacingTheangiographyofcoronarysinusPositionofthelead27CRTindicationsNYHAclassIIorIVheartfailureandintraventricularconductiondelay.

LBBB，LVEF≤35％、QRS≥120ms.

NoneLBBB,LVEF≤35％、QRS≥150ms.Persistentsymptoms

despiteoptimalmedicaltherapywithACEinhibitors,beta-blockers,and/orotherappropriatepharmacologicmeasures.

28Treatmentcontinued

SurgicalCareLeftventricularassistdevicesHearttransplantation29Hypertrophiccardiomyopathy，HCMageneticdisorderthattypicallyisinheritedinanautosomaldominantfashionwithvariableexpressivity.Characteristicoftheasymmetricmyocardialhypertrophy,theobstructionofLVoutflowtractandtheabnormaldiastolicfunction.30SynonymsIdiopathichypertrophicsubaorticstenosis(IHSS);asymmetricseptalhypertrophy(ASH).31EpidemiologyReportedin0.5%oftheoutpatientpopulationreferredforechocardiography.estimatedtooccurin0.05-0.2%ofthepopulation.

Reported1.3/100,000inJiangsu,China.Slightlymorecommoninmalesthaninfemales.inspiteoftheautosomaldominantpattern.Theratioisabout1.6:1inChina.32EpidemiologyAgeanyage.mayshowitselfduring20-30yearsold.Suddendeathinchildren.Familyhistoryapproximately25%offirst-degreerelativesofpatientswithHCMsuffered.33Etiology1.Geneticcausesthecommonmutated-genelocatedonthelongarmofchromosome14,encodingforbetacardiacmyosinheavychainandchromosome11,encodingforcardiacmyosinbindingprotein-C.autosomaldominantMendelian-inheritedwayinabout50%ofcases.sporadiconebyspontaneousmutations.phenotypicexpressionofagivenmutationisvarious.34Etiology

con2.abnormalcalciumkineticsabnormalcalciumfluxes,anincreaseinintracellularcalciumconcentration,increaseinthenumberofcalciumchannels,producehypertrophyandcellulardisarray.

3.OthercausesAbnormalsympatheticstimulation

Abnormallythickenedintramuralcoronaryarteries

Subendocardialischemia

Cardiacstructuralabnormalities

35PathologyAsymmetricseptalhypertrophy(ASH)Septalwallthicknessisfrom20mmto52mm.Myocardialcellhypertrophyanddisarray.

Theabnormalintramuralcoronaryarteries.Theabnormalmitralvalvesetup.36Fig1HCM37Fig2MicroscopicsectionofmyocardiuminHCMmyocytedisarray.CurrProblCardiol2004;29:233-91.38PathophysiologyDynamicpressuregradientacrosstheLVoutflowtract.furthernarrowingofanalreadysmalloutflowtract.Systolicanteriormotion(SAM)ofthemitralvalveagainstthehypertrophiedseptum.VenturieffectAbnormaldiastolicfunction.impairsventricularfillingandincreasesfillingpressure.

SubendocardialischemiaArrhythmia.39Fig3SchematicdrawingofHCMinsystole.

Mitralleafletisdistortedtowardseptum(SAM)resultinginoutflowtractobstructionand

posteriorlydirectedmitralregurgitation.

CurrProblCardiol2004;29:233-91.40SymptomDyspnea

mostcommon.aconsequenceofelevatedLVdiastolicfillingpressures.SyncopeorPresyncopeinadequatecardiacoutputuponexertionorfromcardiacarrhythmia.highriskofsuddendeath.Suddencardiacdeathhighestincidenceinpreadolescentandadolescentchildren,relatedtoextremeexertion.80%ofcasesareduetoventricularfibrillation.41SymptomconAngina

nodetectablecoronaryatherosclerosis.Frommarkedlyincreasedmyocardialoxygenconsumption.Palpitations

andDizziness

common,fromarrhythmias.Congestiveheartfailureinthelatestage.42SignSystolicejectionmurmurbestheardbetweentheapexandleftsternalborder.diminishwithanyincreaseinpreload(eg,Muellermaneuver,squatting),afterload(eg,handgrip)ordecreaseinheartcontractility.increasewithanydecreaseinpreload(eg,Valsalvamaneuver,nitrateadministration,diureticadministration,standing),afterload(eg,vasodilatoradministration)orincreaseinheartcontractility.43AuxiliaryexaminationEchocardiographySeptalhypertrophywithseptal-to-freewallratiogreaterthan1.4:1(1.3~1.5:1).Aabnormalsystolicanteriorleafletmotionofthemitralvalve.ThenarrowingoftheLVoutflowtractDecreasedmidaorticflow,andpartialsystolicclosureoftheaorticvalveinmidsystole.44Two-dimensionalechocardiography

inHCMAsymmetrichypertrophyIVSLVPW45SAMinHCMCDCD46DopplerechocardiograminHCMThemixedcolorintheLVoutflowtract.47CardiacImaging

conElectrocardiography

ST-TwaveabnormalitiesandLVhypertrophy.Differentiatedfromcoronaryheartdisease.

Heartcatheter

the

pressuregradientbetweenLVandoutflowtractisabove30mmHg。48TheECGchangesinHCMST-TandQwavechanges49DiagnosisEchocardiogramcanidentify.

50Treatment

MedicationBeta-adrenergicblockingagents

Propranolol,MetoprololCalciumchannelblockers

Verapamil,Diltiazem

Antiarrhythmics

Amiodarone51MedicationWarningAvoidinotropicdrugsifpossibleAvoidnitratesandsympathomimeticamines,exceptinconcomitantcoronaryarterydiseaseAvoiddigitalisUsediureticswithcaution52TreatmentLeftventricularmyomectomythebenefitisusuallysustained.Documenteddramaticgappressure.PacemakerimplantationDualchamberpacing.TheRVseptalpreexcitationleadstoa"pullingaway"oftheseptumfromtheoutflowregion,allowingforadecreaseinLVoutflowtractobstruction.ICDmayneeded.Catheterseptalablationatherapeuticinfarctionoftheproximalinterventricularseptalmyocardium.Analogoustoasurgicalmyomectomy.53FurtheroutpatientcareAvoidstrenuousexercise.Avoidinappropriatemedicationusage.Carefullymonitormedicationdoseandadverseeffects.Diminishthelikelihoodofsuddendeath.54Thanks

foryourattention!55Myocarditis56DefinitionMyocarditisisdefinedclinicallyasinflammation

oftheheartmuscle.withawiderangeofclinicalpresentation,fromsubtletodevastating.57EpidemiologyAsymptomatic,sofrequencyisdifficulttoascertain.Incidenceusuallyisestimatedat1-10per100,000persons.Accordingtoestimates,asmanyas1-5%ofpatientswithacuteviralinfectionsmayhaveinvolvementofthemyocardium.58EpidemiologyconSex:Incidenceissimilarbetweenmalesandfemales,althoughyoungmalesareparticularlysusceptible.Age:Patientsusuallyarefairlyyoung.Themedianageofpatientsaffectedwithlymphocyticmyocarditisis42years.59EtiologyInfectionViralBacterial,rickettsial,spirochetalProtozoal,MetazoalFungalToxicanthracyclines,catecholamines,Interleukin-2,alpha2interferonHypersensitivityDrugs60PathogenesisDirectcytotoxiceffectofthecausativeagentSecondaryimmuneresponse,whichcanbetriggeredbythecausativeagentCytokineexpressioninthemyocardium(eg,tumornecrosisfactor-alpha,nitricoxidesynthase)4.Aberrantinductionofapoptosis

61ClinicalManifestationsclinicallysilentAntecedentflulikesymptomsaccompaniedbyfever,arthralgia,andmalaise.heartfailuresymptomsChestpain,palpitation,syncope,cardiacshock.AVblockorventriculararrhythmiaSuddendeath62AuxiliaryexaminationElevatedcardiacenzymes(MYO,CKorCK-MB,AST,LDH).Elevatedcardiactroponin(cTn-IorcTn-T)

89%specificityand34%sensitivityandincreasesmorefrequentlythancreatinekinaseMBsubunits.63Auxiliaryexamination

Electrocardiogram

SinustachycardiaismostcommonDiffuseST-TwavechangesVentriculararrhythmiasConductiondelay,evencompleteheartblockcausingAdams-Stokesattack.64Auxiliaryexamination

EchocardiographyLVsystolicdysfunctioniscommonwithsegmentalwallmotionabnormalitiesLVsizeistypicallynormalormildlydilatedWallthicknessmaybeincreasedVentricularthrombidetectedin15%65Auxiliaryexamination

CardiacangiographyToruleout

coronaryischemiaasacauseofnew-onsetheartfailure,especiallywhenclinicalpresentationmimicsacutemyocardialinfarction.66Auxiliaryexamination

RightventricularendomyocardialbiopsyCriterionstandardfordiagnosisofmyocarditis,althoughlimitedsensitivityandspecificity,asinflammationcanbediffuseorfocal.TheDallascriteriaforbiopsy-baseddiagnosisestablishedin1987,

consistofmyocytenecrosisandinflammation.

67Viralmyocarditis:lymphocyte-richinfiltrate

68Giant-cellmyocarditisDiffuseinfiltrationwithnumerousgiantcells,lymphocytes,neutrophils,andeosinophils.Myocytedamageisalsopresent.

69DiagnosisAcutemyocarditisshouldbesuspectedwheneverapatient,especiallyayoungmale,presentswithotherwiseunexplainedcardiacabnormalitiesofnewonset,suchasheartfailure,arrhythmias,orconductiondisturbances.Ahistoryofrecentupperrespiratoryinfectionorenteritismayalsobepresent.70DiagnosisconCongenital,valvular,ischemic,andpulmonaryheartdiseaseshouldberuledoutWhenitcomestothepresumptivediagnosisofcardiomyopathy.Shouldbemadeonthebasisoftheclinicalandlaboratorypresentations.Thedefinitivediagnosisofmyocarditiscanbemadeonlybyendomyocardialbiopsy.71TreatmentMajorityofpatientshaveaself-limiteddisease.Supportivecareisthefirstlineoftherapy.Bedrest.72TreatmentconManagementofLVdysfunctionConsideranticoagulationtopreventthromboembolia.ConsidertemporarypacerforcompleteAVblockIntensiveimmunosuppressivetherapy(eg,corticosteroids,azathioprine,cyclosporine).uncertaineffect.73FollowupRecoverthoroughly.RemainedECGchanges.DysfunctionofLVCardiomyopathyDeath74Ending心包炎心包由壁層和臟層組成的一個潛在腔隙，即心包腔，其間有少量液體（﹤50mL)定義及分類心包炎是指由多種致病因素引起的心包臟層和壁層的炎性病變按病程心包炎可分為急性心包炎、亞急性滲出性縮窄性心包炎、慢性心包積液和慢性縮窄性心包炎等

是由多種原因所致的心包臟層和壁層的急性炎癥性纖維化反應以胸痛、心包摩擦音、特異性心電圖表現(xiàn)為特征急性心包炎（AcutePericarditis）病因

一、特發(fā)性：國外多見

二、感染性：細菌、病毒、結核、真菌、原蟲和艾滋相關性等，我國結核多見。三、腫瘤：原發(fā)性（如間皮細胞瘤）或繼發(fā)性（乳腺、肺、淋巴瘤等）。四、免疫性：RA、SLE、硬皮病、急性風濕熱、皮肌炎等。

病因

五、炎性反應性：心梗后、外傷、外科

手術、放射性、藥物性等。六、代謝性：尿毒癥、痛風、黏液性水

腫等。七、鄰近器官疾病：胸膜炎、主動脈夾

層、肺梗死等。病理

一、早期：心包的臟層和壁層間滲出纖維蛋白和白細胞等。稱為纖維蛋白性或干性心包炎。二、進展期：滲出物中液體增加，液量可多至2～3L，稱為滲出性或濕性心包炎。滲液的性質可為纖維蛋白性、漿液血性或化膿性等。

三、后期：滲出物可完全吸收；或某些心包炎機化為瘢痕甚至鈣化，最終發(fā)展成為縮窄性心包炎。

纖維蛋白性心包炎病理生理

1.滲液慢，心包可逐漸伸展，致使心包壓力不增加，可以無癥狀。積液使心包腔內壓力逐漸上升，當達到一定程度就限制心臟擴張。

2.滲液快，心包壓力急劇增加，心室舒張期充盈減少，導致靜脈壓增加，心搏量降低，血壓下降。

機體代償機制：心肌收縮力增加、心率加快、周圍小動脈阻力增加以維持血壓。

病理生理

3.如心包滲液繼續(xù)增加，心包腔內壓力進一步提高達到右房右室舒張壓水平，其壓差等于零時，心臟壓塞或心包填塞（cardiactamponade）即可發(fā)生。此時代償機制衰竭，即升高的靜脈壓不能增加心室的充盈；過快的心率使心室舒張期縮短和充盈減少，而致心排血量下降；小動脈收縮達到極限，動脈壓下降至循環(huán)衰竭，而產生心源性休克。心臟壓塞是否發(fā)生，取決于心包積液的容量、速度及心包本身的韌性及心肌功能狀態(tài)。10臨床表現(xiàn)—纖維蛋白性心包炎癥狀：主要癥狀：心前區(qū)疼痛為多見疼痛性質：可尖銳，也可呈壓榨性與呼吸運動有關，常因咳嗽、深呼吸、變換體位或吞咽而加重可放射至頸部、左肩、左臂等體征：心包摩擦音為典型體征心前區(qū)的抓刮樣粗噪音與心房收縮、心室收縮和心室舒張相一致的雙相音積液增多時摩擦音消失臨床表現(xiàn)—滲出性心包炎癥狀：呼吸困難是最突出的癥狀，嚴重者端坐呼吸，身體前傾，可有發(fā)紺，或壓迫癥狀：干咳、聲音嘶啞，吞咽困難體征：觸診：心尖搏動弱；叩診：心濁音界向兩側擴大，Ewart征；聽診：心率快，心音遙遠收縮壓降低，舒張壓變化不大，脈壓變??；心臟壓塞嚴重時可出現(xiàn)奇脈出現(xiàn)頸靜脈怒張、肝腫大及下肢水腫

臨床表現(xiàn)—心臟壓塞

急性心臟壓塞，以心輸出量下降肺靜脈壓增高肺循環(huán)淤血為主要表現(xiàn)：心排血量下降、心動過速、紫紺、脈壓變小、收縮壓下降甚至休克

Beck三聯(lián)征：血壓突然下降或休克，頸靜脈顯著怒張，心音低弱遙遠慢性心臟壓塞，以靜脈壓增高，體循環(huán)淤血為主要表現(xiàn)

實驗室檢查1.胸部X線：﹥250ml時，心影開始增大，“燒瓶狀”，搏動減弱或消失。實驗室檢查

2.心電圖改變：ST段抬高，T波改變，PR段壓低急性心包炎急性心肌梗死實驗室檢查

3.超聲心動圖檢查：簡便、安全、靈敏

和可靠。液性暗區(qū)，可確診。實驗室檢查

4.心包穿刺：1）涂片、細菌培養(yǎng)、查找腫瘤細胞和滲液的分類等，有助于確定病因。2）抽