Ebstein畸形的外科治疗

Ebstein畸形的外科治疗第1页/共51页Ebstein畸形是罕见复杂的心脏先天畸形发生率1:40,000-200,000先天性心脏病中:<1%疾病谱宽：

轻型无症状重症新生儿期死亡率极高手术死亡率高WilhelmEbstein1866年首先描述形态HelenTaussig1950年描述临床特点第2页/共51页解剖学特点DisplacementoftheseptalandposteriorleafletsoftheTVtowardtheapexoftheRV.Althoughtheanteriorleafletisattachedattheappropriatelevelofthetricuspidannulus,itislargerthannormalandmayhavemultiplechordalattachmentstotheventricularwall.第3页/共51页3.ThesegmentoftheRVfromthelevelofthetruetricuspidannulustothelevelofattachmentoftheseptalandposteriorleafletsisunusuallythinanddysplastic.ThetricuspidannulusandtheRAareextremelydilated.4.ThecavityofthefunctionalRVisreducedinsize,usuallylacksaninletchamber,andhasasmalltrabecularcomponent.

第4页/共51页5.Theinfundibulumisoftenobstructedbytheredundanttissueoftheanteriorleafletaswellasbythechordalattachmentsoftheanteriorleaflettotheinfundibulum.第5页/共51页临床分型（分级）typeA:thevolumeofthetrueRVisadequate.typeB：thereisalargeatrializedcomponentoftheRV,buttheanteriorleafletmovesfreely.typeC:theanteriorleafletisseverelyrestrictedinitsmovementandmaycausesignficantobstructionoftheRVOT.typeD:thereisalmostcompleteatrializationoftheventriclewiththeexceptionofasmallinfundibularcomponent.Theonlycommunicationbetweentheatrializedventricleandtheinfundibulumisthroughtheanteroseptalcommissureofthetricuspidvalve.第6页/共51页超声评估分级面积比值＝右房＋房化右室/功能右室＋左心房室心脏舒张期四腔心轴面

1级：<＝0.52级：0.6-1.03级：1.1-1.54级：>1.5第7页/共51页病理生理特点：1.三尖瓣关闭不全

右房明显扩大，卵圆孔右向左分流，右室扩大2.右室功能不良

有效收缩部分减少，心室膨胀3.肺动脉发育不良

三尖瓣前叶、乳头肌阻挡，生理性PAA4.左室受压，呈“夹心饼”，功能受限5.可伴有室上性或室性心律

第8页/共51页临床表现：容易疲劳，活动后呼吸困难、心悸，紫绀Giuliani67例非手术，12年观察：

39%NYHA1-2级

61%NYHA3-4级21%病人死亡死亡病人有一项或多项特点：

1.NYHA3-4级

2.心胸比大于0.653.发绀或动脉氧饱和90%以下

4.明确诊断时处于婴儿阶段第9页/共51页术前基础治疗：1.保持PDA开放，增加肺内血供，改善氧合：PGE12.纠正酸中毒3.充分镇静，过度通气，降低肺血管阻力第10页/共51页治疗原则：1.尽可能恢复三尖瓣功能2.右房减容，改善呼吸功能3.根据右室功能决定：

双心室矫治右室旷置右室减负荷4.房化心室是否去除（折叠或切除）？5.右室流出道充分疏通第11页/共51页外科技术：三尖瓣成形（包括心室成形）技术

1.Danielson修复

2.改良Carpentier技术

3.Devega技术

4.前叶单瓣技术第12页/共51页三尖瓣成形技术1.Danielson修复Ebstein畸形的治疗第13页/共51页2.改良Carpentier修复

Ebstein畸形的外科治疗第14页/共51页3.改良Devega技术runingbothendsofthepledgettedsutureinandoutalongtheannulusseparatingtheatrializedfromthefunctionalrightventriclefrom"A"to"B"theanteriorleafletisnotlargeoriftheposteriorleafletiswelldevelopedorifboththeanteriorandposteriorleafletsarefunctionalbutdysplastic

The“playitwhereitlies”approachinvolveslimitedplicationofthetricuspidvalve.PointsAandBareapproximatedwith1or2mattresssuturesatthelevelofthenativevalve,nottothelevelofthetruetricuspidannulus.Thisresultsinapproximatingtheapicalaspectsoftheseptalandanteriorleaflets,effectivelycreatingabicuspidvalve.第15页/共51页4.前瓣单叶修复Ebstein畸形的外科治疗第16页/共51页重症Ebstein畸形的定义目前不明确参考标准

PredictorsofDeathinneonateswithEbstein’sAnomaly

cardiothoracicrationgreaterthan0.85(100%fatal)Echocardiographyscoregrade4/4(100%fatal)Echocardiographyscoregrade¾andcyanosis(100%fatal)Severetricuspidregurgitation(mostlyfatal)Echocardiographyscoregrade¾(45%fatalininfancy)Knott-CraigCJetal.AnnThoracSurg2002,76:1786第17页/共51页新生儿Ebstein畸形的治疗Starnes矫治(JThoracCardiovascSurg1991:101;1082-7)

5consecutivenewborninfants

Age:1-9days.

Weight:3.6±1.8kgMeanPH:7.2±0.05Meanoxygentension:29.6±2.3mmHgMeancardiothoracicration:0.81±0.02ECHO:severetricuspidregurgitationfunctionalpulmonaryatresiainallpatients

Allpatientswereresuscitatedwithintubationandmechanicalventilation,acidosiswascorrected,andtherapywithPGE1.第18页/共51页PreoperativeechoassessmentpatientNo.12345RVdysplasia++00+tetheredanteriorleaflet00+0+Echoscoreratio1.30.90.80.61.01severeTR+++++functionalpulmonaryatresia

+++++第19页/共51页Cardiaccatheterizationassessmentinoneneonates第20页/共51页Operativetechnique

Thetricuspidorificewasclosedwithautologouspericardium.ThecoronarysinusbeneaththepatchtoreducetheriskofAVblock.AnASDwascreatedtoensuremixingattheatriallevel.第21页/共51页Therightatriumwasreducedinsizebyremovingasegmentoftherightatrialfreewall.AA-Pshuntwasestablishedwitha4mmGore-Texvessel.第22页/共51页ResultsNoperioperativeandlatedeaths.Nopostoperativearrhythmias.Mechanicalventilationtime10.2±0.3days.

Po2:42.2±0.9mmHg,SO2:83.2±1.9%第23页/共51页Follow-upOnereceivedaGlennoperationafter11mo.TworeceivedFontanproceduresat23and22moofage.第24页/共51页双心室矫治(Knott-CraigCJ.

RepairofEbstein’sanomalyinthesymptomaticneonate:anevolutionoftechniquewith7-yearfollow-up.AnnThoracSurg2002:73;1786-93)

8symptomaticpatients6neonates(2-19d,2.8-3.2kg)1younginfant(2mo,3.8kg)hadundergoneastarnesoperationelsewhere1infant(4mo,6.4kg)新生儿Ebstein畸形的治疗第25页/共51页PreoperativeassessmentSevere(4/4)TRwaspresentinallexcept1(Starnesoperation)Cardiothoracicratioexceeded0.85inallpatientsEchocardiographyseverityscoreswere>1.5in6(grade4/4)and1.3in1(grade3/4)

3patientshadanatomicalPA2hadfunctionalPA

新生儿Ebstein畸形的治疗第26页/共51页OperativetechniqueRepairconsistedof

TVrepairReductionatrioplastyReliefofRVOTobstructionPartialclosureofASDCorrectionofallassociatedcardiacdefects新生儿Ebstein畸形的治疗第27页/共51页Tricuspidvalverepair(

3hadDanielson-typerepairs,3hadDeVega-typerepairs,and2hadcomplexrepairs)

1.modifiedDanielsontechnique

placingapledgettedsutureattheA-PcommissureandbringingthisdowntotheCS,thuscreatinga"doubleorifice"valve.

ThelateralorificecontainingtheatrializedRV,whichbeclosedbyplicatingitvertically.Ifthelargeanteriorleafletdoesnotcoaptwellwiththeventricularseptum,apledgettedsuturefromtheanteriorpapillarymuscletotheventricularseptummaybeusedtocorrectthis新生儿Ebstein畸形的治疗第28页/共51页2.DeVega-typeannuloplasty

(theanteriorleafletisnotlargeoriftheposteriorleafletiswelldevelopedorifboththeanteriorandposteriorleafletsarefunctionalbutdysplastic)

runingbothendsofthepledgettedsutureinandoutalongtheannulusseparatingtheatrializedfromthefunctionalrightventriclefrom"A"to"B"新生儿Ebstein畸形的治疗第29页/共51页InthemoresevereformsofEAintheneonate

1.TheorificeoftheTVistowardtheapexoftheRV.2.Thecommissurebetweentheanteriorandseptalleafletsmaybeimperforateorpatentonlythroughsmallfenestrations.3.Theposteriorleafletmaybereasonablywelldevelopedandmobile.新生儿Ebstein畸形的治疗第30页/共51页DetachingtheentireanteriorandposteriorleafletsfromtheannulusFreeingtheleafletsfromtheirmuscularizedattachmentsandreducingtheannulusinsizeposteriorlyReattachingtheleafletstothesmallerannulusnotonlycorrectsthedefectbutalsoeffectivelychangestheorientationoftheTVbacktotheRVOTandthefunctionalRV.FenestratingtheA-Scommissureandleafletpreventstricuspidstenosis

第31页/共51页Correctionofallassociatedcardiacdefects

PA、PSorRVOTS:

RVOTpatchorasmallhomograftorothervalvedconduit

VSD:morecomplex

UnloadingtheRV

FenestratedASDclosureAddingthehemi-Fontanconnection(inolderpatients)ReductionatrioplastyOpenrightpleuralcavityandleaveadrainintheperitonealcavity

新生儿Ebstein畸形的治疗第32页/共51页ResultsOnepatientdiedinhospitalnolatedeathsAllareinsinusrhythmandinfunctionalclassI4patientshadtracetomildTRand2hadmildtomoderateregurgitation第33页/共51页外科矫治新观点（SunilP.MalhotraMD,SelectiveRightVentricularUnloadingandNovelTechnicalConceptsinEbstein‘sAnomalys,

SanFrancisco,CA,Jan26–28,2009.

）Newconecpts:

Usingofvalvereconstructivetechniquesthatdiffersubstantiallyfromthoseintheliterature:1A“playitwhereitlies”approachtothetricuspidvalveinwhichthereconstructionisperformedatthefunctionalorificeinsteadofmovingthevalvetotheanatomictricuspidannulus;2Avoidanceofdetachmentandreimplantationofvalveleaflets;and3AlimitedplicationperformedonlyatthelevelofthedisplacedvalveratherthancompleteplicationoftheentireatrializedRV.第34页/共51页Newconecpts:DependingspecificphysiologicandanatomiccriteriaforselectiveuseoftheBDGinconjunctionwithrepairofEbstein'sanomaly.第35页/共51页PatientCharacteristics

93.12-08.1257consecutivepatientsoutsideoftheneonatalperiod

ThediagnosisofsevereEbstein'sanomalyofthetricuspidvalvewasestablishedbyechocardiographyinallpatients.Echocardiographywasusedtocharacterizethedegreeofapicaldisplacementofthetricuspidannulus,theseverityandnatureofTR,andthedegreeofmobilityoftheanteriorleaflet.TRwasclassifiedonascaleof1to4(1,trace;2,mild;3,moderate,and4,severe).Echocardiographyalsowasusedtoassessrightandleftventricularfunctionandtoidentifyanyatriallevelshunts.第36页/共51页PatientCharacteristicsAge:7monthsto40.4yearsexerciseintolerancein40cyanosisin26RVfailurein18atrialdysrhythmiasin8TRwasmoderateorseverein50patients(87.7%).第37页/共51页ApproachestotheTricuspidValve

1Thedetrimentaleffectsofaverylargetricuspidannulus

第38页/共51页ApproachestotheTricuspidValve2ThegoalofplicationoftheatrializedRV

The“playitwhereitlies”approachinvolveslimitedplicationofthetricuspidvalve.PointsAandBareapproximatedwith1or2mattresssuturesatthelevelofthenativevalve,nottothelevelofthetruetricuspidannulus.Thisresultsinapproximatingtheapicalaspectsoftheseptalandanteriorleaflets,effectivelycreatingabicuspidvalve.第39页/共51页3SelectiveuseoftheBDG—usingtheBDGintwoseparateandindependentcircumstances.

Thefirstisphysiologic.CyanosisatrestisamarkerforaninadequateRVpump.Ifthepatientisfullysaturatedatrestbutbecomescyanoticwithexercise,thisisarelativemarkerofaninadequateRVpump,andwewillhavealowthresholdforplacingaBDG.Typically,wewillseparatethepatientfromcardiopulmonarybypassaftervalverepairandmonitorrightandleftatrialpressure.Iftherightatrialpressureexceeds1.5timestheleftatrialpressureundertheserelativelyunstressedconditionsofanopenchestinananesthetizedpatient,wewillperformaBDG.Ifthepatientpresentswithanintactatrialseptumoranatrialseptaldefectwithleft-to-rightshunting,aBDGisnotperformed.第40页/共51页ThesecondcircumstanceforplacingaBDGisanatomicandrelatestotheultimatesizeofthefunctionaltricuspidannulusafterrepair.Ifitisnecessarytomakethefunctionaltricuspidorificesubstantiallylessthan2.5cm(ina70-kgpatient)toachieveacompetentvalve,wewillassessinflowvelocityacrossthetricuspidafterseparationfromcardiopulmonarybypassusingtransesophagealechocardiography.Ifobstructionisdemonstrated,aBDGisplaced.Weacknowledgethatmanyofthemaneuversusedtomakearegurgitantvalvecompetentinvolvereducingthevalveopening.ThisoptionforBDGusefreesustoaggressivelyreducethefunctionalvalveorificeasmuchasnecessarytoachieveastable,competentvalverepair.第41页/共51页ConcomitantProceduresPerformedatInitialEbstein'sAnomalyRepair

ProceduresNo.

Electrophysiologicprocedures8

Ablationofaccessorypathway2

Maze

procedures

Bilateral2

Withpacemaker1

Right-sided3

Withpacemaker1

Pacemakeralone1

Partialanomalouspulmonaryveinrepair1Pulmonaryvalvereplacement1ReliefofRVoutflowtractobstruction2Supravalvarpulmonarystenosisrepair1第42页/共51页Results

Noearlyorlatedeathsoccurred.Earlyreoperationwasrequiredin2patients.1patientrequiredpacemakerplacementforatrioventricularnodalblockand1patientrequiredplacementofanCRTforrecurrentventriculararrhythmias.Atfo