ebstein畸形的外科治療醫(yī)學(xué)幻燈片

1、Ebstein畸形的外科治療策略Ebstein畸形是罕見復(fù)雜的心臟先天畸形發(fā)生率1:40000200000先天性心臟病中:1%疾病譜寬：輕型無癥狀重癥新生兒期死亡率極高手術(shù)死亡率高WilhelmEbstein1866年首先描述形態(tài)HelenTaussig1950年描述臨床特點解剖學(xué)特點DisplacementoftheseptalposterileafletsoftheTVtowardtheapexoftheRV.Althoughthe

2、anterileafletisattachedattheappropriatelevelofthetricuspidannulusitislargerthannmalmayhavemultiplechdalattachmentstotheventricularwall.3.ThesegmentoftheRVfromthelevelofthetruetricuspidannulustothelevelofattachmentofthese

3、ptalposterileafletsisunusuallythindysplastic.ThetricuspidannulustheRAareextremelydilated.4.ThecavityofthefunctionalRVisreducedinsizeusuallylacksaninletchamberhasasmalltrabecularcomponent.5.Theinfundibulumisoftenobstructe

4、dbytheredundanttissueoftheanterileafletaswellasbythechdalattachmentsoftheanterileaflettotheinfundibulum.臨床分型（分級）typeA:thevolumeofthetrueRVisadequate.typeB：thereisalargeatrializedcomponentoftheRVbuttheanterileafletmovesfr

5、eely.typeC:theanterileafletisseverelyrestrictedinitsmovementmaycausesignficantobstructionoftheRVOT.typeD:thereisalmostcompleteatrializationoftheventriclewiththeexceptionofasmallinfundibularcomponent.Theonlycommunicationb

6、etweentheatrializedventricletheinfundibulumisthroughtheanteroseptalcommissureofthetricuspidvalve.超聲評估分級面積比值＝右房＋房化右室功能右室＋左心房室心臟舒張期四腔心軸面1級：1.5病理生理特點：臨床表現(xiàn)：容易疲勞，活動后呼吸困難、心悸，紫紺Giuliani67例非手術(shù)，12年觀察：39%NYHA12級61%NYHA34級21%病人死亡死亡

7、病人有一項或多項特點：1.NYHA34級2.心胸比大于0.653.發(fā)紺或動脈氧飽和90%以下4.明確診斷時處于嬰兒階段術(shù)前基礎(chǔ)治療：1.保持PDA開放，增加肺內(nèi)血供，改善氧合：PGE12.糾正酸中毒3.充分鎮(zhèn)靜，過度通氣，降低肺血管阻力治療原則：1.盡可能恢復(fù)三尖瓣功能2.右房減容，改善呼吸功能3.根據(jù)右室功能決定：雙心室矯治右室曠置右室減負(fù)荷4.房化心室是否去除（折疊或切除）？5.右室流出道充分疏通外科技術(shù)：三尖瓣成形（包括心室成形）

8、技術(shù)1.Danielson修復(fù)2.改良Carpentier技術(shù)3.Devega技術(shù)4.前葉單瓣技術(shù)三尖瓣成形技術(shù)1.Danielson修復(fù)Ebstein畸形的治療2.改良Carpentier修復(fù)Ebstein畸形的外科治療3.改良Devega技術(shù)runingbothendsofthepledgettedsutureinoutalongtheannulusseparatingtheatrializedfromthefunctionalri

9、ghtventriclefromAtoBtheanterileafletisnotlargeiftheposterileafletiswelldevelopedifboththeanteriposterileafletsarefunctionalbutdysplasticThe“playitwhereitlies”approachinvolveslimitedplicationofthetricuspidvalve.PointsABar

10、eapproximatedwith12mattresssuturesatthelevelofthenativevalvenottothelevelofthetruetricuspidannulus.Thisresultsinapproximatingtheapicalaspectsoftheseptalanterileafletseffectivelycreatingabicuspidvalve.4.前瓣單葉修復(fù)Ebstein畸形的外科

11、治療重癥Ebstein畸形的定義目前不明確參考標(biāo)準(zhǔn)PredictsofDeathinneonateswithEbstein’sAnomalycardiothacicrationgreaterthan0.85(100%fatal)Echocardiographyscegrade44(100%fatal)Echocardiographyscegradecyanosis(100%fatal)Severetricuspidregurgitati

12、on(mostlyfatal)Echocardiographyscegrade(45%fatalininfancy)KnottCraigCJetal.AnnThacSurg200276:1786新生兒Ebstein畸形的治療Starnes矯治(JThacCardiovSurg1991:10110827)5consecutivenewbninfantsAge:19days.Weight:3.61.8kgMeanPH:7.20.05Mean

13、oxygentension:29.62.3mmHgMeancardiothacicration:0.810.02ECHO:severetricuspidregurgitationfunctionalpulmonaryatresiainallpatientsAllpatientswereresuscitatedwithintubationmechanicalventilationacidosiswascrectedtherapywithP

14、GE1.PreoperativeechoassessmentpatientNo.12345RVdysplasia00tetheredanterileaflet000Echosceratio1.30.90.80.61.01severeTRfunctionalpulmonaryatresiaCardiaccatheterizationassessmentinoneneonatesOperativetechniqueThetricuspidi

15、ficewasclosedwithautologouspericardium.TheconarysinusbeneaththepatchtoreducetheriskofAVblock.AnASDwascreatedtoensuremixingattheatriallevel.Therightatriumwasreducedinsizebyremovingasegmentoftherightatrialfreewall.AAPshunt

16、wasestablishedwitha4mmGeTexvessel.ResultsNoperioperativelatedeaths.Nopostoperativearrhythmias.Mechanicalventilationtime10.20.3days.Po2:42.20.9mmHgSO2:83.21.9%FollowupOnereceivedaGlennoperationafter11mo.TworeceivedFontanp

17、roceduresat2322moofage.雙心室矯治(KnottCraigCJ.RepairofEbstein’sanomalyinthesymptomaticneonate:anevolutionoftechniquewith7yearfollowup.AnnThacSurg2002:73178693)8symptomaticpatients6neonates(219d2.83.2kg)1younginfant(2mo3.8kg)

18、hadundergoneastarnesoperationelsewhere1infant(4mo6.4kg)新生兒Ebstein畸形的治療PreoperativeassessmentSevere(44)TRwaspresentinallexcept1(Starnesoperation)Cardiothacicratioexceeded0.85inallpatientsEchocardiographyseveritysceswere1.

19、5in6(grade44)1.3in1(grade34)3patientshadanatomicalPA2hadfunctionalPA新生兒Ebstein畸形的治療OperativetechniqueRepairconsistedofTVrepairReductionatrioplastyReliefofRVOTobstructionPartialclosureofASDCrectionofallassociatedcardiacde

20、fects新生兒Ebstein畸形的治療Tricuspidvalverepair(3hadDanielsontyperepairs3hadDeVegatyperepairs2hadcomplexrepairs)1.modifiedDanielsontechniqueplacingapledgettedsutureattheAPcommissurebringingthisdowntotheCSthuscreatingadoubleific

21、evalve.ThelateralificecontainingtheatrializedRVwhichbeclosedbyplicatingitvertically.Ifthelargeanterileafletdoesnotcoaptwellwiththeventricularseptumapledgettedsuturefromtheanteripapillarymuscletotheventricularseptummaybeu

22、sedtocrectthis新生兒Ebstein畸形的治療2.DeVegatypeannuloplasty(theanterileafletisnotlargeiftheposterileafletiswelldevelopedifboththeanteriposterileafletsarefunctionalbutdysplastic)runingbothendsofthepledgettedsutureinoutalongthea

23、nnulusseparatingtheatrializedfromthefunctionalrightventriclefromAtoB新生兒Ebstein畸形的治療InthemeseverefmsofEAintheneonate1.TheificeoftheTVistowardtheapexoftheRV.2.Thecommissurebetweentheanteriseptalleafletsmaybeimperfatepatent

24、onlythroughsmallfenestrations.3.Theposterileafletmaybereasonablywelldevelopedmobile.新生兒Ebstein畸形的治療DetachingtheentireanteriposterileafletsfromtheannulusFreeingtheleafletsfromtheirmuscularizedattachmentsreducingtheannulus

25、insizeposterilyReattachingtheleafletstothesmallerannulusnotonlycrectsthedefectbutalsoeffectivelychangestheientationoftheTVbacktotheRVOTthefunctionalRV.FenestratingtheAScommissureleafletpreventstricuspidstenosisCrectionof

26、allassociatedcardiacdefectsPA、PSRVOTS:RVOTpatchasmallhomograftothervalvedconduitVSD:mecomplexUnloadingtheRVFenestratedASDclosureAddingthehemiFontanconnection(inolderpatients)ReductionatrioplastyOpenrightpleuralcavityleav

27、eadrainintheperitonealcavity新生兒Ebstein畸形的治療ResultsOnepatientdiedinhospitalnolatedeathsAllareinsinusrhythminfunctionalclassI4patientshadtracetomildTR2hadmildtomoderateregurgitation外科矯治新觀點（SunilP.MalhotraMDiveRightVentricu

28、larUnloadingNovelTechnicalConceptsinEbstein‘sAnomalysSanFranciscoCAJan26–282009.）Newconecpts:Usingofvalvereconstructivetechniquesthatdiffersubstantiallyfromthoseintheliterature:1A“playitwhereitlies”approachtothetricuspid

29、valveinwhichthereconstructionisperfmedatthefunctionalificeinsteadofmovingthevalvetotheanatomictricuspidannulus2Avoidanceofdetachmentreimplantationofvalveleaflets3Alimitedplicationperfmedonlyatthelevelofthedisplacedvalver

30、atherthancompleteplicationoftheentireatrializedRV.Newconecpts:DependingspecificphysiologicanatomiccriteriafiveuseoftheBDGinconjunctionwithrepairofEbsteinsanomaly.Patientacteristics93.1208.1257consecutivepatientsoutsideof

31、theneonatalperiodThediagnosisofsevereEbsteinsanomalyofthetricuspidvalvewasestablishedbyechocardiographyinallpatients.EchocardiographywasusedtoacterizethedegreeofapicaldisplacementofthetricuspidannulustheseveritynatureofT

32、Rthedegreeofmobilityoftheanterileaflet.TRwasclassifiedonascaleof1to4(1trace2mild3moderate4severe).Echocardiographyalsowasusedtoassessrightleftventricularfunctiontoidentifyanyatriallevelshunts.PatientacteristicsAge:7month

33、sto40.4yearsexerciseintolerancein40cyanosisin26RVfailurein18atrialdysrhythmiasin8TRwasmoderateseverein50patients(87.7%).ApproachestotheTricuspidValve1ThedetrimentaleffectsofaverylargetricuspidannulusApproachestotheTricus

34、pidValve2ThegoalofplicationoftheatrializedRVThe“playitwhereitlies”approachinvolveslimitedplicationofthetricuspidvalve.PointsABareapproximatedwith12mattresssuturesatthelevelofthenativevalvenottothelevelofthetruetricuspida

35、nnulus.Thisresultsinapproximatingtheapicalaspectsoftheseptalanterileafletseffectivelycreatingabicuspidvalve.3iveuseoftheBDG—usingtheBDGintwoseparateindependentcircumstances.Thefirstisphysiologic.Cyanosisatrestisamarkerfa

36、ninadequateRVpump.IfthepatientisfullysaturatedatrestbutbecomescyanoticwithexercisethisisarelativemarkerofaninadequateRVpumpwewillhavealowthresholdfplacingaBDG.Typicallywewillseparatethepatientfromcardiopulmonarybypassaft

37、ervalverepairmonitrightleftatrialpressure.Iftherightatrialpressureexceeds1.5timestheleftatrialpressureundertheserelativelyunstressedconditionsofanopenchestinananesthetizedpatientwewillperfmaBDG.Ifthepatientpresentswithan

38、intactatrialseptumanatrialseptaldefectwithlefttorightshuntingaBDGisnotperfmed.ThesecondcircumstancefplacingaBDGisanatomicrelatestotheultimatesizeofthefunctionaltricuspidannulusafterrepair.Ifitisnecessarytomakethefunction

39、altricuspidificesubstantiallylessthan2.5cm(ina70kgpatient)toachieveacompetentvalvewewillassessinflowvelocityacrossthetricuspidafterseparationfromcardiopulmonarybypassusingtransesophagealechocardiography.Ifobstructionisde

40、monstratedaBDGisplaced.Weacknowledgethatmanyofthemaneuversusedtomakearegurgitantvalvecompetentinvolvereducingthevalveopening.ThisoptionfBDGusefreesustoaggressivelyreducethefunctionalvalveificeasmuchasnecessarytoachieveas

41、tablecompetentvalverepair.ConcomitantProceduresPerfmedatInitialEbsteinsAnomalyRepairProceduresNo.Electrophysiologicprocedures8Ablationofaccessypathway2MazeproceduresBilateral2Withpacemaker1Rightsided3Withpacemaker1Pacema

42、keralone1Partialanomalouspulmonaryveinrepair1Pulmonaryvalvereplacement1ReliefofRVoutflowtractobstruction2Supravalvarpulmonarystenosisrepair1ResultsNoearlylatedeathsoccurred.Earlyreoperationwasrequiredin2patients.1patient

43、requiredpacemakerplacementfatrioventricularnodalblock1patientrequiredplacementofanCRTfrecurrentventriculararrhythmias.AtfollowupechocardiographyRVsystolicfunctionwasnmalin52patientsmildlyreducedin3moderatelyreducedin2pat

44、ients.Severityoftricuspidregurgitationisshownbefeafterrepair.NYHAstatusimprovedfrom2.30.5preoperativelyto1.00.2atfollowup(p=0.0002).Outcomesof“OneaHalfVentricle”Repairs31casespre:SO289.5%5.9%vs96.2%3.9%p=0.01NYHA2.50.6vs

45、2.160.4p=0.0025TherewerenoBDGrelatedcomplicationsThemeansaturationwas96.9%3.0%NYHAfunctionalstatusinthiscohtimprovedto1.00.2(p=0.0002).我院情況1996年10月－2005年10月151例，死亡7例心胸比0.726例術(shù)前TV均為大量返流5月60歲，8.358kg死亡5例，2例ECMO（均存活）手術(shù)方法單純G

46、lenn（常溫1例）3例Glenn＋Danielson成形1例Glenn＋Carpentier成形1例Glenn＋Devega環(huán)縮1例死亡全腔改Glenn1例死亡TVR（包括TVR＋Glenn1例）5例死亡3例Danielson成形（包括RVOTS疏通2例)6例Carpentier成形5例Devega環(huán)縮3例2例ECMO術(shù)后早期TV返流，右心功能嚴(yán)重衰竭，左心受累右心EF:20－25%、10－15％ECMO支持時間8天、5天右心明顯縮小