应激性心肌病.ppt

Stress-InducedCardiomyopathy(Tako-tsubosyndrome)應激性心肌病,澳門鏡湖醫院心內科金椿,病情介紹,女性，70歲(住院號：08-4361)主訴：胸痛1小時。AED(2008.2.2821:45)：BP156/84mmHg,HR90bpmEKG,2008.2.2821:54,2008.2.2823:37,2008.2.2908:13,心肌酶譜變化,病情介紹,入院查體：BP127/70mmHg，HR82bpm既往史：10多年前因右腎結石行右腎切除術，1991年曾患十二指腸潰瘍。不嗜煙酒。有上感史多天。入院診斷：ACS？,MildstenosisatmidLAD;Mitralregurgitation.,Coronaryangiogram,Leftventriculogram,PoorEF(42%)withhypokinesisoftheanteriorwallofLV.,Echocardiogram,Onemonthlater,住院期間,BP100-130/60-80mmHgX-Ray：輕度肺瘀血；心影橫徑增大(c/t=0.63)，左室大。Holter：極偶發室上性早摶部分成對出現血生化：TG2.0mmol/L，HDL1.1mmol/L，血糖、肝腎功能正常出院帶藥：Betaloc-zok12.5mgqdAcertil2mgqdAspirin0.1qdVasteralMR35mgbidNMR2.5mgbid,病史小結,危險因素：絶經期後女性，血脂升高誘因：因丈夫去世而情緒激動表現：胸痛30min檢查：心電圖有動態變化，血心肌酶CAG：冠脈無明顯狹窄Echo：心尖摶動瀰漫性減弱，各房室不大住院期間：生命體征平穩，無心衰及心律失常,Stress-InducedCardiomyopathy(Tako-tsubosyndrome)應激性心肌病,In1990HikaruSatoandcolleaguesfromJapandescribedanovelcardiacsyndrome,characterisedby:transientleftventriculardysfunctionwithchestpain,ECGchangesminimalreleaseofmyocardialenzymesmimickinganAMILeftventriculogramrevealed:leftventriclehadapeculiarshape(aroundbottomandnarrowneck)resembledatypeofbottleusedinJapanfortrappingoctopus.SatoandcolleaguestermedthesyndromeTako-tsubocardiomyopathy“tako”meaningoctopus,and“tsubo”,bottle.,LVventricularangiogramwithtypicalapicalballooning.,Morerecently,ithasalsobeencalled:acuteleftventricularballooningreversiblestresscardiomyopathybrokenheartsyndromestress-inducedmyocardialstunningApicalballooningsyndrome,StressappearstobekeytothedevelopmentofTako-tsubo:canbeemotional,physicalorpsychologicalinnatureStudiesshowTako-tsubohasoccurred:afterearthquakesdeathofarelativecaraccidentssurprisepartiesfierceargumentscourtappearancesandarmedrobberies,Clinicalfeatures,Chestpainisthemostcommonsymptom-upto90%dyspnoeapalpitationssyncopeAswithAMIfeaturesofhighcirculatingadrenalinelevels(suchasdiaphoresisandperipheralshutdown)arealsocommonRequiringIABPcounterpulsationandmechanicalventilation(1-5%)Othercomplicationsarerare:leftventricularthrombusformation,ventricularruptureandintractablearrhythmias,ECGchanges,ECGchangesonadmissionareoftenindistinguishablefromacuteanteriormyocardialinfarctionSTelevation,usuallyinV3V6,withevolvingT-waveinversion,Laterinthecourse(after3days),widespreaddeepT-waveinversionisoftenseenwithsignificantQTprolongation,Cardiacbiomarker,Serialtroponinandck-MBlevelsonlyasmallrisethisisanimportantdifferencefromAMI.Asmallproportionofpatientswillhavenotroponinriseatall,andtheabsenceofelevationdoesnotexcludethediagnosis.,Coronaryangiography,UponadmissioncoronaryangiographyrevealednooronlyadiffuseCADwithoutobstructivestenoses(50%),orspontaneousvasospasminallpatients,Leftventriculography,akinesiaintheanterolateral,apical,diaphragmatic,septalareasaswellasbasehypercontractileThemedianEFoftheLVwas30.4%.,End-diastolicandend-systolicframesoftheLV(AandB)andRV(CandD)demonstratingextentofLVandRVdysfunction(arrows).,Echocardiogram,ApicaltwochamberechocardiographicviewshowingLVapicalballooningandsigmoidseptum,End-diastolicandend-systolicapicalfour-and-twochamberechocardiographicviewsdemonstratingthetypicalapicalandmid-ventricularLVwall-motionabnormalitiesofapatientwithtakotsubocardiomyopathy,14studies:2%ofSTelevationinfarcts,mostcasesinpost-menopausalwomen.chestpainanddyspnoeain67.8and17.8%Cardiogenicshock(4.2%)ventricularfibrillation(1.5%)ST-segmentelevation(81.6%)Twaveabnormalities(64.3%)Qwaves(31.8%)Cardiacbiomarkersmildlyelevated(86.2%)LVdysfunctiononadmissionEF20to49%,overaperiodofdaystoweeks.precededbyemotional(26.8%)orphysicalstress(37.8%).Norepinephrineconcentrationwaselevated(74.3%)excellent,withfullrecoveryinmostpatients.In-hospitalmortalitywas1.1%.Only3.5%ofthepatientsexperiencedarecurrence.,Comparisonbetweenpositronemissiontomography(A,C,andE)andsingle-photonemissioncomputedtomography(B,D,andF)images:metabolicimagerevealedseverelyreducedF-18fluorodeoxyglucoseuptakeintheapicalandmid-ventricularsegmentscomparedwithperfusionabnormalities.(AandB)Horizontallong-axis;(CandD)verticallong-axis;(EandF)short-axis.,Lightmicroscopy,Endomyocardialbiopsyspecimen:contraction-bandnecrosis(arrows)andsmallamountsofmononuclearcellinfiltration(haematoxylinandeosinstain).(A)Originalmagnificationx100;(B)originalmagnificationx200.,PASstaining(arrows)showsremarkableintracellularaccumulationofglycogen(A).Afterfunctionalrecoveryonlysmallamountsofglycogenparticularlyaroundthenucleiofmyocytes(arrows)weredocumented(B).,Electronmicroscopy,Electronmicroscopyofacutebiopsiesshowingnumerousvacuolesofdifferentsizesandcontents(myelinbodies,residualcellularproducts),lossofcontractilematerial,andareasofnon-specifiedcytoplasm(A).Theinterstitialspacewaswidenedcontainingformationofcellulardebris(B).Intheacutephase,formationofmyelinbodiescouldbedocumented(C).InTTCcontractionbandsofsarcomereswerefound(D).Recoveredbiopsiesshowedanearlycompleterearrangementofcontractilematerialwithregularlydistributedsarcomeres,normalnuclei,andmitochondria(E,F).vac,vacuole;svac,smallvacuoles;N,nucleus;cyt,cytoplasm;mit,mitochondria;cd,cellulardebris;mb,myeliesbodies;sarc,sarcomeres;cb,contractionband.,Immunohistochemistry,Immunohistochemistryofintracellularproteins(specificlabellinggreen,phalloidinred,nucleiblue).-actininwasdetectedonlyintheborderzoneduringTTC(A).Afterfunctionalrecoveryaregulardistributionwasfound(B).N-terminaldystrophinshowedadecreaseinTTCverifyingalossofprotein-to-proteininteraction(C)incomparisonwithbiopsiesafterfunctionalrecovery(D).C-terminaldystrophinwasunalteredinTTCsuggestingthatintegrityofthesarcolemmaismaintained(E,F).Connexin-43showedareducedcellcellconnectioninTTC(G),whereasamyocardialintegritywasdocumentedafterfunctionalrecovery(H).,ImmunolabellingfortitinwasperformedusingT12(A,B)andTz1/Z2(C,D).F-actin(red)wasvisualizedwithTRITCconjugatedphalloidinandnuclei(blue)werecounterstainedwithDraq-5.Notethattitinintheacutestage(A,C)iseitherabsentinthecentralpartsofthemyocytesorshowsapunctuatedpatternascomparedwithaclearcross-striatedpatternoflabellingandhigherexpressionlevelsintherecoveryphase(B,D).,Immunohistochemistryofextracellularproteins(specificlabellinggreen,phalloidinred,nucleiblue).TheECMstainedbyfibronectin(A,B)andcollagen-1(C,D)wasincreasedandthemyocardialsyncytiumwasseparated.Afterfunctionalrecovery,adecreaseofextracellularproteinswasobserved.Macrophages(arrows)showinginflammatoryresponsewereregionallyaccumulatedinTTC(E,F).SlightincreaseofT-lymphocytes(arrows)wasregionallyobservedinTTC(G,H).,Pathophysiology,precisemechanismsareunknowncatecholamine-mediatedmechanismswithlikelymediationviacardiacsympatheticnerves.Suddensurgingcatecholaminelevels,canbeprecipitatedbyemotionalorphysicalstressCatecholaminelevelsarecharacteristicallyfarhigherthaninmatchedpatients,catecholamine-mediatedmultivesselepicardialspasm,microvascularcoronaryspasm,orpossibledirectcatecholamine-mediatedmyocyteinjury.,Pathophysiology,Pathophysiology,Onmyocardialbiopsy,thehistologicalappearancesareverysimilartocontractionbandnecrosisseeninphaeochromocytomaInarodentmodel,TTCcanbepreventedwith-or-blockadeThemoredensedistributionofadrenoceptorsattheapexmightexplainwhytheapexisaffectedwhilethebaseissparedInaddition,oestrogendownregulatescardiacadrenoceptorsandattenuatestheirresponsetoactivation,providing