内科学教学课件：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心包炎心包由壁层和脏层组成的一个潜在腔隙，即心包腔，其间有少量液体（﹤50mL)定义及分类心包炎是指由多种致病因素引起的心包脏层和壁层的炎性病变按病程心包炎可分为急性心包炎、亚急性渗出性缩窄性心包炎、慢性心包积液和慢性缩窄性心包炎等

是由多种原因所致的心包脏层和壁层的急性炎症性纤维化反应以胸痛、心包摩擦音、特异性心电图表现为特征急性心包炎（AcutePericarditis）病因

一、特发性：国外多见

二、感染性：细菌、病毒、结核、真菌、原虫和艾滋相关性等，我国结核多见。三、肿瘤：原发性（如间皮细胞瘤）或继发性（乳腺、肺、淋巴瘤等）。四、免疫性：RA、SLE、硬皮病、急性风湿热、皮肌炎等。

病因

五、炎性反应性：心梗后、外伤、外科

手术、放射性、药物性等。六、代谢性：尿毒症、痛风、黏液性水

肿等。七、邻近器官疾病：胸膜炎、主动脉夹

层、肺梗死等。病理

一、早期：心包的脏层和壁层间渗出纤维蛋白和白细胞等。称为纤维蛋白性或干性心包炎。二、进展期：渗出物中液体增加，液量可多至2～3L，称为渗出性或湿性心包炎。渗液的性质可为纤维蛋白性、浆液血性或化脓性等。

三、后期：渗出物可完全吸收；或某些心包炎机化为瘢痕甚至钙化，最终发展成为缩窄性心包炎。

纤维蛋白性心包炎病理生理

1.渗液慢，心包可逐渐伸展，致使心包压力不增加，可以无症状。积液使心包腔内压力逐渐上升，当达到一定程度就限制心脏扩张。

2.渗液快，心包压力急剧增加，心室舒张期充盈减少，导致静脉压增加，心搏量降低，血压下降。

机体代偿机制：心肌收缩力增加、心率加快、周围小动脉阻力增加以维持血压。

病理生理

3.如心包渗液继续增加，心包腔内压力进一步提高达到右房右室舒张压水平，其压差等于零时，心脏压塞或心包填塞（cardiactamponade）即可发生。此时代偿机制衰竭，即升高的静脉压不能增加心室的充盈；过快的心率使心室舒张期缩短和充盈减少，而致心排血量下降；小动脉收缩达到极限，动脉压下降至循环衰竭，而产生心源性休克。心脏压塞是否发生，取决于心包积液的容量、速度及心包本身的韧性及心肌功能状态。10临床表现—纤维蛋白性心包炎症状：主要症状：心前区疼痛为多见疼痛性质：可尖锐，也可呈压榨性与呼吸运动有关，常因咳嗽、深呼吸、变换体位或吞咽而加重可放射至颈部、左肩、左臂等体征：心包摩擦音为典型体征心前区的抓刮样粗噪音与心房收缩、心室收缩和心室舒张相一致的双相音积液增多时摩擦音消失临床表现—渗出性心包炎症状：呼吸困难是最突出的症状，严重者端坐呼吸，身体前倾，可有发绀，或压迫症状：干咳、声音嘶哑，吞咽困难体征：触诊：心尖搏动弱；叩诊：心浊音界向两侧扩大，Ewart征；听诊：心率快，心音遥远收缩压降低，舒张压变化不大，脉压变小；心脏压塞严重时可出现奇脉出现颈静脉怒张、肝肿大及下肢水肿

临床表现—心脏压塞

急性心脏压塞，以心输出量下降肺静脉压增高肺循环淤血为主要表现：心排血量下降、心动过速、紫绀、脉压变小、收缩压下降甚至休克

Beck三联征：血压突然下降或休克，颈静脉显著怒张，心音低弱遥远慢性心脏压塞，以静脉压增高，体循环淤血为主要表现

实验室检查1.胸部X线：﹥250ml时，心影开始增大，“烧瓶状”，搏动减弱或消失。实验室检查

2.心电图改变：ST段抬高，T波改变，PR段压低急性心包炎急性心肌梗死实验室检查

3.超声心动图检查：简便、安全、灵敏

和可靠。液性暗区，可确诊。实验室检查

4.心包穿刺：1）涂片、细菌培养、查找肿瘤细胞和渗液的分类等，有助于确定病因。2）抽