臨床病理討論會小兒科：盧俊維醫師放射科：吳金珠醫師

病理科：蕭正祥醫師臨床病理討論會A10y/ogirlChiefcomplaint:Chestdiscomfort,vomitinganddrycoughforoneday臨床病理討論會BriefHistoryGrowth&development:Weight:22kg(3rd-10thpercentile)Height:130cm(25-50thpercentile)Developmentmilestone:withinnormallimitPasthistoryHand-foot-mouthdiseasein1998FrequentURIandfeverduringchildhoodNodrugorfoodallergy臨床病理討論會BriefHistoryFamilyhistory:HersisterhadfeverandURIrecently.臨床病理討論會PresentIllnessFeverandbilateralhandarthralgiaattackonce1monthagoChestdiscomfortandcoughsince9/11afternoon,2001VisitLMDandURIwastoldVomitingandchesttightnesson9/120AMand5AM臨床病理討論會PresentIllness9/12morning,visitLMDagain,ECGshowedarrhythmiaReferto亞東hospital臨床病理討論會PresentIllnessFindingsat亞東hospitalClearconsciousness,ill-looking,pallorappearance,nocyanosis

IrregularheartbeatEKG:VPCbigeminy臨床病理討論會PresentIllnessLab.findingsat亞東hospitalWBC9000/mm3,Hb13.5g/dlBUN11mg/dl,Cre0.6mg/dlGOT25U/L,

CK665U/L,CK-MB175U/L臨床病理討論會PresentIllnessEchocardiogramat亞東hospitalMultiplesmallVSDs,musculartrabeculartype,atapexLVdyskinesia,LVEF60-70%MildTR,mildMR臨床病理討論會PresentIllnessManagementat亞東hospitalLidocaineivdripDopamine10mg/kg/minRefertoNTUH(2pm)臨床病理討論會PhysicalExaminationPhysicalfindingsatNTUHConsciousness:lethargic,acuteill-lookingT/P/R:37/140/25BP80/46SaO297%HEENT:paleconjunctiva

anictericscleramildcyanoticlip臨床病理討論會PhysicalExaminationNeck:jugularvenousengorgementChest:bilateralbasalr?lesHeart:irregularlyirregularbeats,distantheartsoundnomurmur臨床病理討論會PhysicalExaminationAbdomen:nohepatomegalyhypoactivebowelsoundExtremities:freelymovablecoldandcyanoticpoorcapillaryrefilling臨床病理討論會InitialLabDataCBC:WBCHbHctPlt884012.7

37.2%160KSeg82.4%,Lym13.8%,Eos0.1%BCS:BUNCreNaKClCa12.80.631414.51042.41

臨床病理討論會InitialLabDataVBG:pHpCO2pO2HCO3BE7.3647.427.326.9+1.4Cardiacenzyme:

CPK(U/L)CK-MBTroponinI(ng/ml)1040196.5

31.9CRP:0.53mg/dl

臨床病理討論會InitialLabDataEKG(9/12):

臨床病理討論會InitialLabDataEKG(9/12):

臨床病理討論會InitialLabDataEKG(9/12):

臨床病理討論會InitialLabDataEchocardiogram(9/12):LVenlargementLVEF45%MuscularVSDMildMR,TR,PR

臨床病理討論會Echocardiogram(9/12)臨床病理討論會CourseandTreatmentManagementForcardiogenicshock:Dopamine,Dobutamin,Primacor,LasixForventriculararrhythmia:Amiodarone,Lidocaine,MgSO4Formyocarditis:IVIG,Considerextracorporealmembranousoxygenator(ECMO)support臨床病理討論會CourseandTreatment9/125pm(3hrafteradmission)ProgressivehypotensionSuddenonsetofcoma,BPdrop(pulseless)EKG:ventriculartachycardiaStartCPR(40min)StartECMO,transfertoSICU臨床病理討論會EKG(9/12,5PM)臨床病理討論會CourseinSICUECMOsettingV-AECMO:15FrR’tfemoralartery,19FrR’tfemoralveinbycutdownFlow:2000ml/minMeanBP:70mmHgUrineoutput:1.72ml/kg/hr臨床病理討論會Echocardiogram(9/13)臨床病理討論會CourseinSICUVTpersistentdespiteofcardioversion,Lidocaine,Amiodarone,MgSO49/12~9/17:ECMO5daysPoorLVfunctionPersistentlungedema(CXR,clinically)TnIslowlydecreaseA-lineflatten,nopulsatilewaveform臨床病理討論會CourseinSICUEndomyocardialbiopsy(9/14)MildtomoderateperivascularandinterstitiallymphocyteinfiltrationFociofmyocytedegenerationInterstitialedemaNogiantcell

Compatiblewithacutemyocarditis臨床病理討論會CourseinSICULAdrain(9/17):TodecompressLV,avoidthrombosisLAdomecannulation

connectingtoFVcannulaECMOFALAP:22mmHg10mmHg臨床病理討論會Echocardiogram(9/17)臨床病理討論會CourseinSICU9/18,4amAcutethrombosisatLAcannulaandECMOcircuit

poorflowCPRfor30min.andemergentre-setECMOtubingCons.AfterCPR:E1M1VTLightreflex(+)臨床病理討論會CourseinSICU9/19,8am:grosshematuriaandECMOtubethrombosis

resetECMOProgressivedilatedpupils,nolightreflex,suspectedhypoxicencephalopathyRemoveECMOon9/23(10thday)臨床病理討論會Labdata9/129/139/149/159/169/17TnI31.962.4>1007437.3CK104091242342126759138647026CK-MB196368687403207101Cre0.630.590.560.50.470.51Bil1.240.510.651.361.51.35臨床病理討論會LabData臨床病理討論會LabDataSerologystudy;MycoplasmapneumoniaIgM:(9/12)positive,(9/21)negativeOthervirologystudy:allnegativeCoxsackieA,CoxsackieB1-B6,CMVIgG&IgM,Enterovirus70,InfluenzaA&B臨床病理討論會LabDataCulture:Throatswab(9/12):StaphylococcusaureusNasalswab(9/12):Staphylococcusaureus,ViridansstreptococciBlood(9/19):Staphylococcusepidermidis臨床病理討論會DiscussionDiagnosticapproach:CauseofchestpaininchildrenIdiopathic:12-45%Costochondritis:9-22%Musculoskeletaltrauma:21%Cough,asthma,pneumonia:15-21%Psychogenicfactors:5-9%GIdisorders:4-7%Cardiacdisorders:0-4%臨床病理討論會DiagnosticapproachHx:cough,vomitingPE:hypotensionjugularvenousdistentiontachycardiairregularheartbeatbasalr?lespoorperipheralperfusionCardiovascularcompromise

臨床病理討論會DiagnosticapproachFlu-likeillness,arrhythmia,cardiovascularcompromiseAcutemyocarditishighlysuspectedD/D:DilatedcardiomyopathyAnomalousleftcoronaryarteryChronictachyarrhythmiaPericarditis

臨床病理討論會DiagnosticapproachEKG:VPCbigeminy,ventriculartachycardiaST-segmentchangeElevatedcardiacenzymeEchocardiogram:markedLVdyskinesiaEndomyocardialbiopsyLymphocyteinfiltrationMyocytedegeneration

Acutemyocarditisconfirmed臨床病理討論會ClinicalclassificationofmyocarditisFulminantAcuteChronicactiveChronicpersistentInitialpresentationShock,severeLVdysfuntionCHFCHFNormalLVfunctionEndomyocardialbiopsyMultifocalactivemyocarditisActiveorborderlinemyocarditisActiveorborderlinemyocarditisActiveorborderlinemyocarditisNaturehistoryCompleterecoveryordeathIncompleterecoveryorDCMDCMNormalLVfunction臨床病理討論會Myocarditis:anenigmaticdisease!臨床病理討論會DarksideofthemyocarditisInitialnon-specificsymptoms

DifficulttoestablishthediagnosisEtiologyhardtofindComplexityofpathogenesisOftenrefractorytoconventionaltreatment臨床病理討論會DarksideofthemyocarditisInitialnon-specificsymptoms

Similartopatientswithsepsis,bronchiolitis,pneumonia,gastroenteritis,hepatitis,andrenalfailureetc.AggressivefluidresuscitationmayharmunstablepatientsRapidprogressioninfulminantmyocarditis臨床病理討論會DarksideofthemyocarditisDifficulttoestablishthediagnosisLimitedsensitivityandspecificityofchangesinCXR,ECG,cardiacenzyme(Troponinlevel:moresensitive)Echocardiogram:LVdysfunction,oftenregionalEndomyocardialbiopsy:asgoldstandard,butsensitivity3-63%臨床病理討論會DallascriteriaBorderlinemyocarditisActivemyocarditisAmJCadiovascPathol1987;1:3-14臨床病理討論會DarksideofthemyocarditisEtiologyhardtofindVIRALCAUSESEnterovirusCoxsackieACoxsackieB

EchovirusPoliovirusAdenovirusCytomegalovirusHerpesvirusInfluenzaAEpstein-BarrvirusVaricellaMumpsMeaslesParvovirusRabiesHepatitisB,CRubellaRubeolaRespiratorysyncytialvirusHumanimmunodeficiencyvirusRickettsialRickettsiaricketsiiRickettsiatsutsugamushiBacterialMeningococcusKlebsiellaLeptospiraMycoplasmaSalmonellaClostridiaTuberculosisBrucellaLegionellapneumophilasmallpoxStreptococcusProtozoalTrypanosomacruziToxoplasmosisAmebiasisOtherparasitesToxocaracanisSchistosomiasisHetereophyiasisCysticercosisEchinococcusViscerallarvamigransTrichinosisFungiandyeasts

ActinomycosisCoccidiodomycosisHistoplasmosisCandidaNONVIRALCAUSES臨床病理討論會DarksideofthemyocarditisEtiologyhardtofindToxicScorpionDiphtheriaDrugsSulfonamidesPhenylbutazoneCyclophosphamideNeomercazoleAcetazolamideAmphotericinBIndomethacinTetracyclineIsoniazidMethyldopaPhenytoinPenicillinHypersensitivity/Autoimmune

Rheumatoidarthritis

Rheumaticfever

UlcerativecolitisSystemiclupuserythematosusMixedconnectivetissuediseaseSclerodermaWhipple'sdiseaseOtherSarcoidosis

Kawasakidisease

CornstarchNONINFECTIOUSETIOLOGIES臨床病理討論會DarksideofthemyocarditisEtiologyhardtofindPediatrCardiol2001;22:34-9臨床病理討論會DarksideofthemyocarditisComplexityofpathogenesisNEJM2000;343:1388-98臨床病理討論會DarksideofthemyocarditisComplexityofpathogenesisFactorscontributingtohostsusceptibilityAutoantibodies:toadenosinenucleotidetranslocator,myosinExpressionofcelladhesionmolecules(ICAM-1)Expressionofcoxsackie-adenovirusreceptor(CAR)臨床病理討論會DarksideofthemyocarditisOftenrefractorytoconventionaltreatmentStandardtherapy:ACEinhibitor,inotropicagents,diuretics–oftennoteffectiveinfulminantmyocarditisImmunosuppression:IVIG,steroids,cyclosporin–stillcontroversial臨床病理討論會BrightsideofthemyocarditisGoodlongtermprognosisoffulminantmyocarditisImprovementofmechanicalsupport:LVAD,BVAD,ECMO臨床病理討論會BrightsideofthemyocarditisGoodlongtermprognosisoffulminantmyocarditisNEJM2000;342:690-5臨床病理討論會BrightsideofthemyocarditisGoodlongtermprognosisoffulminantmyocarditis臨床病理討論會Brightsideofthemyocardit