EbsteinEbstein畸形的外科治疗策略EbsteinEbstein畸形是罕见复杂的心畸形是罕见复杂的心脏先天畸形脏先天畸形l l发生率发生率1:40,000-200,000l l先天性心脏病中先天性心脏病中: 1%l l疾病谱宽：疾病谱宽： 轻型轻型轻型轻型 无症状无症状无症状无症状 重症重症重症重症 新生儿期死亡率极高新生儿期死亡率极高新生儿期死亡率极高新生儿期死亡率极高 手术死亡率高手术死亡率高手术死亡率高手术死亡率高Wilhelm Ebstein 1866 年首先描述形态Helen Taussig 1950 年描述临床特点解剖学特点解剖学特点1. 1.DisplacementoftheseptalandposteriorleafletsoftheTVtowardtheapexoftheRV.2. 2.Althoughtheanteriorleafletisattachedattheappropriatelevelofthetricuspidannulus,itislargerthannormalandmayhavemultiplechordalattachmentstotheventricularwall.3.ThesegmentoftheRVfromthelevelofthe3.ThesegmentoftheRVfromthelevelofthetruetricuspidannulustotheleveloftruetricuspidannulustothelevelofattachmentoftheattachmentoftheseptalseptalandposteriorandposteriorleafletsisunusuallythinandleafletsisunusuallythinanddysplasticdysplastic.The.ThetricuspidannulusandtheRAareextremelytricuspidannulusandtheRAareextremelydilated.dilated.4.ThecavityofthefunctionalRVisreducedin4.ThecavityofthefunctionalRVisreducedinsize,usuallylacksaninletchamber,andhasasize,usuallylacksaninletchamber,andhasasmallsmalltrabeculartrabecularcomponent.component. 5. Theinfundibulumisoftenobstructedbytheredundanttissueoftheanteriorleafletaswellasbythechordalattachmentsoftheanteriorleaflettotheinfundibulum.临床分型（分级）临床分型（分级）typeA:typeA:thevolumeofthetrueRVisadequate.thevolumeofthetrueRVisadequate.typeBtypeB：thereisalargethereisalargeatrializedatrializedcomponentofcomponentoftheRV,buttheanteriorleafletmovesfreely.theRV,buttheanteriorleafletmovesfreely.typeC:typeC:theanteriorleafletisseverelyrestrictedinitsmovementandtheanteriorleafletisseverelyrestrictedinitsmovementandmaycausemaycausesignficantsignficantobstructionoftheRVOT.obstructionoftheRVOT.typeD:typeD:thereisalmostcompletethereisalmostcompleteatrializationatrializationoftheventriclewiththeoftheventriclewiththeexceptionofasmallexceptionofasmallinfundibularinfundibular component.Thecomponent.Theonlycommunicationonlycommunicationbetweenthebetweentheatrializedatrializedventricleandtheventricleandtheinfundibuluminfundibulumisthroughtheisthroughtheanteroseptalanteroseptal commissurecommissureofthetricuspidvalve.ofthetricuspidvalve.超声评估分级超声评估分级面积比值右房房化右室面积比值右房房化右室面积比值右房房化右室面积比值右房房化右室/ / / /功能右室左心房室功能右室左心房室功能右室左心房室功能右室左心房室 心脏舒张期四腔心轴面心脏舒张期四腔心轴面心脏舒张期四腔心轴面心脏舒张期四腔心轴面 1 1级：级： 1.5 1.5病理生理特点：病理生理特点：1. 1. 三尖瓣关闭不全三尖瓣关闭不全三尖瓣关闭不全三尖瓣关闭不全 右房明显扩大，卵圆孔右向左分流，右室扩大右房明显扩大，卵圆孔右向左分流，右室扩大右房明显扩大，卵圆孔右向左分流，右室扩大右房明显扩大，卵圆孔右向左分流，右室扩大2. 2. 右室功能不良右室功能不良右室功能不良右室功能不良 有效收缩部分减少，心室膨胀有效收缩部分减少，心室膨胀有效收缩部分减少，心室膨胀有效收缩部分减少，心室膨胀3. 3. 肺动脉发育不良肺动脉发育不良肺动脉发育不良肺动脉发育不良 三尖瓣前叶、乳头肌阻挡，生理性三尖瓣前叶、乳头肌阻挡，生理性三尖瓣前叶、乳头肌阻挡，生理性三尖瓣前叶、乳头肌阻挡，生理性PAAPAAPAAPAA4. 4. 左室受压，呈左室受压，呈左室受压，呈左室受压，呈“ “夹心饼夹心饼夹心饼夹心饼” ”，功能受限，功能受限，功能受限，功能受限5. 5. 可伴有室上性或室性心律可伴有室上性或室性心律可伴有室上性或室性心律可伴有室上性或室性心律 临床表现：临床表现：容易疲劳容易疲劳容易疲劳容易疲劳 ，活动后呼吸困难、心悸，紫绀，活动后呼吸困难、心悸，紫绀，活动后呼吸困难、心悸，紫绀，活动后呼吸困难、心悸，紫绀Giuliani 67Giuliani 67Giuliani 67Giuliani 67例非手术，例非手术，例非手术，例非手术， 12121212年观察：年观察：年观察：年观察： 39% NYHA 1-239% NYHA 1-239% NYHA 1-239% NYHA 1-2级级级级 61% NYHA 3-461% NYHA 3-461% NYHA 3-461% NYHA 3-4级级级级 21%21%21%21%病人死亡病人死亡病人死亡病人死亡死亡病人有一项或多项特点：死亡病人有一项或多项特点：死亡病人有一项或多项特点：死亡病人有一项或多项特点： 1.NYHA 3-41.NYHA 3-41.NYHA 3-41.NYHA 3-4级级级级 2.2.2.2.心胸比大于心胸比大于心胸比大于心胸比大于0.650.650.650.65 3. 3. 3. 3.发绀或动脉氧饱和发绀或动脉氧饱和发绀或动脉氧饱和发绀或动脉氧饱和90%90%90%90%以下以下以下以下 4.4.4.4.明确诊断时处于婴儿阶段明确诊断时处于婴儿阶段明确诊断时处于婴儿阶段明确诊断时处于婴儿阶段术前基础治疗：术前基础治疗：1.1.保持保持PDAPDA开放，增加肺内血供，改善氧开放，增加肺内血供，改善氧合：合：PGE1PGE12.2.纠正酸中毒纠正酸中毒3.3.充分镇静，过度通气，降低肺血管阻力充分镇静，过度通气，降低肺血管阻力治疗原则：治疗原则：1.1.尽可能恢复三尖瓣功能尽可能恢复三尖瓣功能2.2.右房减容，改善呼吸功能右房减容，改善呼吸功能3.3.根据右室功能决定：根据右室功能决定： 双心室矫治双心室矫治双心室矫治双心室矫治 右室旷置右室旷置右室旷置右室旷置 右室减负荷右室减负荷右室减负荷右室减负荷4.4.房化心室是否去除房化心室是否去除（折叠或切除）（折叠或切除）（折叠或切除）（折叠或切除）？5.5.右室流出道充分疏通右室流出道充分疏通外科技术：外科技术：n n三尖瓣成形（包括心室成形）技术三尖瓣成形（包括心室成形）技术 1.Danielson1.Danielson修复修复 2.2.改良改良CarpentierCarpentier技术技术 3.Devega3.Devega技术技术 4.4.前叶单瓣技术前叶单瓣技术U三尖瓣成形技术三尖瓣成形技术1.Danielson 1.Danielson 修复修复EbsteinEbsteinEbsteinEbstein畸形的治疗畸形的治疗畸形的治疗畸形的治疗2.2.改良改良CarpentierCarpentier修复修复 EbsteinEbsteinEbsteinEbstein畸形的外科治疗畸形的外科治疗畸形的外科治疗畸形的外科治疗3.3.改良改良DevegaDevega技术技术runingbothendsofthepledgettedsutureinandoutalongtheannulusseparatingtheatrializedfromthefunctionalrightventriclefromAtoBthe anterior leaflet is not large or if the posterior leaflet is well developed or if both the anterior and posterior leaflets are functional but dysplastic The The “play it where it liesplay it where it lies” approach involves limited approach involves limited plicationplication of the tricuspid valve. Points A and B are approximated of the tricuspid valve. Points A and B are approximated with 1 or 2 mattress sutures at the level of the native valve, with 1 or 2 mattress sutures at the level of the native valve, not to the level of the true tricuspid annulus. This results in not to the level of the true tricuspid annulus. This results in approximating the apical aspects of the approximating the apical aspects of the septalseptal and anterior and anterior leaflets, effectively creating a bicuspid valve. leaflets, effectively creating a bicuspid valve. 4.4.前瓣单叶修复前瓣单叶修复EbsteinEbsteinEbsteinEbstein畸形的外科治疗畸形的外科治疗畸形的外科治疗畸形的外科治疗重症重症EbsteinEbstein畸形的定义畸形的定义n n目前不明确目前不明确目前不明确目前不明确n n参考标准参考标准参考标准参考标准 PredictorsofDeathinneonateswithPredictorsofDeathinneonateswithEbsteinsEbsteinsAnomalyAnomaly cardiothoracicrationgreaterthan0.85(100%fatal)cardiothoracicrationgreaterthan0.85(100%fatal)Echocardiographyscoregrade4/4(100%fatal)Echocardiographyscoregrade4/4(100%fatal)Echocardiographyscoregradeandcyanosis(100%fatal)Echocardiographyscoregradeandcyanosis(100%fatal)Severetricuspidregurgitation(mostlyfatal)Severetricuspidregurgitation(mostlyfatal)Echocardiographyscoregrade(45%fatalininfancy)Echocardiographyscoregrade(45%fatalininfancy)Knott-CraigCJetal.AnnKnott-CraigCJetal.AnnThoracThorac SurgSurg2002,76:17862002,76:1786新生儿新生儿EbsteinEbstein畸形的治疗畸形的治疗& Starnes矫治矫治(J(JThoracThorac CardiovascCardiovasc SurgSurg1991:101;1082-7)1991:101;1082-7) 5consecutivenewborninfants5consecutivenewborninfants Age:1-9days.Age:1-9days.Weight:3.6Weight:3.61.8kg1.8kgMeanPH:7.20.05MeanPH:7.20.05Meanoxygentension:29.62.3mmHgMeanoxygentension:29.62.3mmHgMeancardiothoracicration:0.810.02Meancardiothoracicration:0.810.02ECHO:severetricuspidregurgitationECHO:severetricuspidregurgitationfunctionalpulmonaryfunctionalpulmonaryatresiaatresiainallpatientsinallpatientsAllpatientswereresuscitatedwithAllpatientswereresuscitatedwithintubationintubationandmechanicalandmechanicalventilation,acidosiswascorrected,andtherapywithPGE1.ventilation,acidosiswascorrected,andtherapywithPGE1.U UPreoperativeechoassessmentPreoperativeechoassessmentpatientNo.patientNo.1234512345RVRVdysplasiadysplasia+00+00+tetheredanteriorleaflettetheredanteriorleaflet00+0+00+0+Echoscoreratio1.30.90.80.61.01Echoscoreratio1.30.90.80.61.01severeTRsevereTR+functionalpulmonaryfunctionalpulmonaryatresiaatresia +U UCardiaccatheterizationassessmentinoneneonatesCardiaccatheterizationassessmentinoneneonatesU UOperativetechniqueOperativetechnique UThe tricuspid orifice was closed with autologous pericardium.UThe coronary sinus beneath the patch to reduce the risk of AV block.UAn ASD was created to ensure mixing at the atrial level.The right atrium was reduced in size by removing a segment of the right atrial free wall.A A-P shunt was established with a 4mm Gore-Tex vessel.UResultsNoNoperioperativeperioperativeandlatedeaths.andlatedeaths.Nopostoperativearrhythmias.Nopostoperativearrhythmias.Mechanicalventilationtime10.2Mechanicalventilationtime10.20.3days.0.3days. PoPo2 2:42.2:42.20.9mmHg,SO0.9mmHg,SO2 2:83.21.9%:83.21.9%UFollow-upOnereceivedaGlennoperationafter11mo.TworeceivedFontanproceduresat23and22moofage.&双心室矫治双心室矫治( (Knott-CraigCJ.Knott-CraigCJ. Repair of Repair of EbsteinsEbsteins anomaly in the symptomatic neonate: an evolution of technique with anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up .7-year follow-up .AnnAnnThoracThorac SurgSurg2002:73;1786-93)2002:73;1786-93)8symptomaticpatients8symptomaticpatients6neonates(2-19d,2.8-3.2kg)6neonates(2-19d,2.8-3.2kg)1younginfant(2mo,3.8kg)hadundergone1younginfant(2mo,3.8kg)hadundergoneaastarnesstarnesoperationelsewhereoperationelsewhere1infant(4mo,6.4kg)1infant(4mo,6.4kg)新生儿新生儿新生儿新生儿EbsteinEbsteinEbsteinEbstein畸形的治疗畸形的治疗畸形的治疗畸形的治疗UPreoperativeassessmentLLSevere(4/4)TRwaspresentinallexcept1Severe(4/4)TRwaspresentinallexcept1(Starnesoperation)(Starnesoperation)LLCardiothoracicratioexceeded0.85inallpatientsCardiothoracicratioexceeded0.85inallpatientsLLEchocardiographyseverityscoreswereEchocardiographyseverityscoreswere1.5in6(grade4/4)1.5in6(grade4/4)and1.3in1(grade3/4)and1.3in1(grade3/4) LL 3 3patientshadanatomicalPApatientshadanatomicalPA2hadfunctionalPA2hadfunctionalPA 新生儿新生儿新生儿新生儿EbsteinEbsteinEbsteinEbstein畸形的治疗畸形的治疗畸形的治疗畸形的治疗UOperativetechniqueRepairconsistedofRepairconsistedof TVrepairTVrepairReductionReductionatrioplastyatrioplastyReliefofRVOTobstructionReliefofRVOTobstructionPartialclosureofASDPartialclosureofASDCorrectionofallassociatedcardiacdefectsCorrectionofallassociatedcardiacdefects新生儿新生儿新生儿新生儿EbsteinEbsteinEbsteinEbstein畸形的治疗畸形的治疗畸形的治疗畸形的治疗U UTricuspidvalverepair( (3hadDanielson-type3hadDanielson-typerepairs,3hadrepairs,3hadDeVegaDeVega-typerepairs,and2hadcomplexrepairs)-typerepairs,and2hadcomplexrepairs) 1.modified Danielson technique placingapledgettedsutureattheA-PcommissureandbringingthisdowntotheCS,thuscreatingadoubleorificevalve. ThelateralorificecontainingtheatrializedRV,whichbeclosedbyplicatingitvertically.Ifthelargeanteriorleafletdoesnotcoaptwellwiththeventricularseptum,apledgettedsuturefromtheanteriorpapillarymuscletotheventricularseptummaybeusedtocorrectthis新生儿新生儿新生儿新生儿EbsteinEbsteinEbsteinEbstein畸形的治疗畸形的治疗畸形的治疗畸形的治疗2.DeVega-typeannuloplasty (the anterior leaflet is not large or if the posterior leaflet is well developed or if both the anterior and posterior leaflets are functional but dysplastic ) ) runingbothendsofthepledgettedsutureinandoutalongtheannulusseparatingtheatrializedfromthefunctionalrightventriclefromAtoB新生儿新生儿新生儿新生儿EbsteinEbsteinEbsteinEbstein畸形的治疗畸形的治疗畸形的治疗畸形的治疗InthemoresevereformsofEAintheneonateInthemoresevereformsofEAintheneonate 1.TheorificeoftheTVistowardtheapex1.TheorificeoftheTVistowardtheapexoftheRV.oftheRV.2.The2.Thecommissurecommissurebetweentheanteriorandbetweentheanteriorand septalseptalleafletsmaybeimperforateorpatentleafletsmaybeimperforateorpatentonlythroughsmallfenestrations.onlythroughsmallfenestrations.3.Theposteriorleafletmaybereasonably3.Theposteriorleafletmaybereasonablywelldevelopedandmobile.welldevelopedandmobile.新生儿新生儿新生儿新生儿EbsteinEbsteinEbsteinEbstein畸形的治疗畸形的治疗畸形的治疗畸形的治疗DetachingtheentireanteriorandposteriorleafletsfromtheannulusFreeingtheleafletsfromtheirmuscularizedattachmentsandreducingtheannulusinsizeposteriorlyReattachingtheleafletstothesmallerannulusnotonlycorrectsthedefectbutalsoeffectivelychangestheorientationoftheTVbacktotheRVOTandthefunctionalRV.FenestratingtheA-Scommissureandleafletpreventstricuspidstenosis U UCorrectionofallassociatedcardiacdefectsCorrectionofallassociatedcardiacdefects PAPA、 PSorRVOTS:PSorRVOTS: RVOTpatchorasmallhomograftorotherRVOTpatchorasmallhomograftorothervalvedvalvedconduitconduit VSD:morecomplexVSD:morecomplex U UUnloadingtheRVUnloadingtheRVFenestratedASDclosureFenestratedASDclosureAddingthehemi-Addingthehemi-FontanFontanconnection(inolderpatients)connection(inolderpatients)U UReductionReductionatrioplastyatrioplastyU UOpenrightpleuralcavityandleaveadrainintheOpenrightpleuralcavityandleaveadrainintheperitonealcavityperitonealcavity 新生儿新生儿新生儿新生儿EbsteinEbsteinEbsteinEbstein畸形的治疗畸形的治疗畸形的治疗畸形的治疗&ResultsJJOnepatientdiedinhospitalOnepatientdiedinhospitalJJnolatedeathsnolatedeathsJJAllareinsinusrhythmandinfunctionalclassIAllareinsinusrhythmandinfunctionalclassIJJ4patientshadtracetomildTRand2hadmildto4patientshadtracetomildTRand2hadmildtomoderateregurgitationmoderateregurgitation&外科矫治新观点外科矫治新观点（Sunil P. Sunil P. MalhotraMalhotra MD, MD, Selective Right Ventricular Unloading and Novel Technical Concepts in Selective Right Ventricular Unloading and Novel Technical Concepts in EbsteinsEbsteins AnomalysAnomalys, , SanFrancisco,CA,Jan2628,2009.SanFrancisco,CA,Jan2628,2009. ）NewNewconecptsconecpts: : UsingofvalvereconstructivetechniquesthatdifferUsingofvalvereconstructivetechniquesthatdiffersubstantiallyfromthoseintheliterature:substantiallyfromthoseintheliterature:1 A “play it where it lies” approach to the tricuspid valve in which the 1 A “play it where it lies” approach to the tricuspid valve in which the reconstruction is performed at the functional orifice instead of reconstruction is performed at the functional orifice instead of moving the valve to the anatomic tricuspid annulus; moving the valve to the anatomic tricuspid annulus; 2 Avoidance of detachment and 2 Avoidance of detachment and reimplantationreimplantation of valve leaflets; and of valve leaflets; and3 A limited 3 A limited plicationplication performed only at the level of the displaced valve performed only at the level of the displaced valve rather than complete rather than complete plicationplication of the entire of the entire atrializedatrialized RV. RV.Newconecpts:DependingspecificphysiologicandanatomicDependingspecificphysiologicandanatomiccriteriaforselectiveuseoftheBDGincriteriaforselectiveuseoftheBDGinconjunctionwithrepairofconjunctionwithrepairofEbsteinsEbsteinsanomaly.anomaly.PatientCharacteristicsPatientCharacteristics 93.12-08.1257consecutivepatientsoutsideofthe93.12-08.1257consecutivepatientsoutsideoftheneonatalperiodneonatalperiod ThediagnosisofsevereEbsteinsanomalyofthetricuspidvalvewasestablishedbyechocardiographyinallpatients.Echocardiographywasusedtocharacterizethedegreeofapicaldisplacementofthetricuspidannulus,theseverityandnatureofTR,andthedegreeofmobilityoftheanteriorleaflet.TRwasclassifiedonascaleof1to4(1,trace;2,mild;3,moderate,and4,severe).Echocardiographyalsowasusedtoassessrightandleftventricularfunctionandtoidentifyanyatriallevelshunts.PatientCharacteristicsPatientCharacteristicsAge:7monthsto40.4yearsAge:7monthsto40.4yearsexerciseintolerancein40exerciseintolerancein40cyanosisin26cyanosisin26RVfailurein18RVfailurein18atrialatrial dysrhythmiasdysrhythmiasin8in8TRwasmoderateorseverein50patients(87.7%).TRwasmoderateorseverein50patients(87.7%).Approaches to the Tricuspid ValveApproaches to the Tricuspid ValveApproaches to the Tricuspid ValveApproaches to the Tricuspid Valve 1 The detrimental effects of a very large tricuspid annulus Approaches to the Tricuspid ValveApproaches to the Tricuspid ValveApproaches to the Tricuspid ValveApproaches to the Tricuspid Valve2 The goal of plication of the atrialized RV The“playitwhereitlies”approachinvolveslimitedplicationofthetricuspidvalve.PointsAandBareapproximatedwith1or2mattresssuturesatthelevelofthenativevalve,nottothelevelofthetruetricuspidannulus.Thisresultsinapproximatingtheapicalaspectsoftheseptalandanteriorleaflets,effectivelycreatingabicuspidvalve.3 3 Selective use of the BDGSelective use of the BDGusing the BDG in two using the BDG in two separate and independent circumstances. separate and independent circumstances. The first is physiologic. Cyanosis at rest is a marker for The first is physiologic. Cyanosis at rest is a marker for an inadequate RV pump. If the patient is fully saturated at an inadequate RV pump. If the patient is fully saturated at rest but becomes cyanotic with exercise, this is a relative rest but becomes cyanotic with exercise, this is a relative marker of an inadequate RV pump, and we will have a low marker of an inadequate RV pump, and we will have a low threshold for placing a BDG. Typically, we will separate the threshold for placing a BDG. Typically, we will separate the patient from cardiopulmonary bypass after valve repair and patient from cardiopulmonary bypass after valve repair and monitor right and left monitor right and left atrialatrial pressure. If the right pressure. If the right atrialatrial pressure exceeds 1.5 times the left pressure exceeds 1.5 times the left atrialatrial pressure under these pressure under these relatively unstressed conditions of an open chest in an relatively unstressed conditions of an open chest in an anesthetized patient, we will perform a BDG.anesthetized patient, we will perform a BDG. If the patient presents with an intact If the patient presents with an intact atrialatrial septum or an septum or an atrialatrial septalseptal defect with left-to-right shunting, a BDG is not defect with left-to-right shunting, a BDG is not performed. performed. ThesecondcircumstanceforplacingaBDGisanatomicThesecondcircumstanceforplacingaBDGisanatomicandrelatestotheultimatesizeofthefunctionaltricuspidandrelatestotheultimatesizeofthefunctionaltricuspidannulusafterrepair.Ifitisnecessarytomakethefunctionalannulusafterrepair.Ifitisnecessarytomakethefunctionaltricuspidorificesubstantiallylessthan2.5cm(ina70-kgtricuspidorificesubstantiallylessthan2.5cm(ina70-kgpatient)toachieveacompetentvalve,wewillassessinflowpatient)toachieveacompetentvalve,wewillassessinflowvelocityacrossthetricuspidafterseparationfromvelocityacrossthetricuspidafterseparationfromcardiopulmonarybypassusingcardiopulmonarybypassusingtransesophagealtransesophageal echocardiography.Ifobstructionisdemonstrated,aBDGisechocardiography.Ifobstructionisdemonstrated,aBDGisplaced.Weacknowledgethatmanyofthemaneuversusedtoplaced.Weacknowledgethatmanyofthemaneuversusedtomakeamakearegurgitantregurgitantvalvecompetentinvolvereducingthevalvevalvecompetentinvolvereducingthevalveopening.ThisoptionforBDGusefreesustoaggressivelyreduceopening.ThisoptionforBDGusefreesustoaggressivelyreducethefunctionalvalveorificeasmuchasnecessarytoachieveathefunctionalvalveorificeasmuchasnecessarytoachieveastable,competentvalverepair.stable,competentvalverepair.ConcomitantProceduresPerformedatInitialConcomitantProceduresPerformedatInitialEbsteinsEbsteinsAnomalyRepairAnomalyRepairProceduresNo.ProceduresNo.ElectrophysiologicElectrophysiologicprocedures8procedures8Ablationofaccessorypathway2Ablationofaccessorypathway2MazeMaze proceduresproceduresBilateraBilateral2l2Withpacemaker1Withpacemaker1Right-sided3Right-sided3Withpacemaker1Withpacemaker1Pacemakeralone1Pacemakeralone1Partialanomalouspulmonaryveinrepair1Partialanomalouspulmonaryveinrepair1Pulmonaryvalvereplacement1Pulmonaryvalvereplacement1ReliefofRVoutflowtractobstruction2ReliefofRVoutflowtractobstruction2SupravalvarSupravalvarpulmonarypulmonarystenosisstenosisrepair1repair1Results Noearlyorlatedeathsoccurred.Noearlyorlatedeathsoccurred.EarlyEarlyreoperationreoperationwasrequiredin2wasrequiredin2patients.patients.1patientrequiredpacemakerplacement1patientrequiredpacemakerplacementforforatrioventricularatrioventricularnodalblockand1patientnodalblockand1patientrequiredplacementofanCRTforrecurrentrequiredplacementofanCRTforrecurrentventriculararrhythmias.ventriculararrhythmias.Atfollow-upechocardiography,RVAtfollow-upechocardiography,RVsystolicfunctionwasnormalin52patients,systolicfunctionwasnormalin52patients,mildlyreducedin3,andmoderatelyreducedmildlyreducedin3,andmoderatelyreducedin2patients.in2patients. Severity of tricuspid regurgitation is shown before and after repair. NYHA status improved from 2.3 0.5 preoperatively to 1.0 0.2 at follow-up (p = 0.0002). Outcomesof“OneandaHalfVentricle”RepairsOutcomesof“OneandaHalfVentricle”Repairs 31 cases pre:SO289.5%5.9%pre:SO289.5%5.9%vsvs96.2%3.9%,96.2%3.9%,p p=0.01=0.01 NYHA2.50.6NYHA2.50.6vsvs2.160.4,2.160.4,p p=0.0025=0.0025 TherewerenoBDG-relatedcomplicationsTherewerenoBDG-relatedcomplicationsThemeansaturationwas96.9%3.0%Themeansaturationwas96.9%3.0%NYHAfunctionalstatusinthiscohortimprovedNYHAfunctionalstatusinthiscohortimprovedto1.00.2(p=0.0002).to1.00.2(p=0.0002).我院情况我院情况U U19961996年年1010月月20052005年年1010月月 151151151151例，死亡例，死亡例，死亡例，死亡7 7 7 7例例例例U U心胸比心胸比0.7,260.7,26例例, ,术前术前TVTV均为大量返流均为大量返流 5 5月月-60-60岁，岁，8.3-58kg8.3-58kg 死亡死亡死亡死亡5 5 5 5例，例，例，例，2 2 2 2例例例例ECMOECMOECMOECMO（均存活）（均存活）（均存活）（均存活）U手术方法手术方法&单纯单纯单纯单纯Glenn Glenn Glenn Glenn （常温（常温（常温（常温1 1 1 1例）例）例）例） 3 3 3 3例例例例&GlennGlennGlennGlennDanielsonDanielsonDanielsonDanielson成形成形成形成形 1 1 1 1例例例例 GlennGlennGlennGlennCarpentierCarpentierCarpentierCarpentier成形成形成形成形 1 1 1 1例例例例 GlennGlennGlennGlennDevegaDevegaDevegaDevega环缩环缩环缩环缩 1 1 1 1例例例例 死亡死亡死亡死亡 全腔改全腔改全腔改全腔改 Glenn 1Glenn 1Glenn 1Glenn 1例例例例 死亡死亡死亡死亡&TVR TVR TVR TVR （包括包括包括包括TVR TVR TVR TVR Glenn 1Glenn 1Glenn 1Glenn 1例例例例） 5 5 5 5例例例例 死亡死亡死亡死亡3 3 3 3例例例例&DanielsonDanielsonDanielsonDanielson成形成形成形成形（包括（包括（包括（包括RVOTSRVOTSRVOTSRVOTS疏通疏通疏通疏通2 2 2 2例例例例) ) ) ) 6 6 6 6例例例例 CarpentierCarpentierCarpentierCarpentier成形成形成形成形 5 5 5 5例例例例 DevegaDevegaDevegaDevega环缩环缩环缩环缩 3 3 3 3例例例例2 2例例ECMOECMO&术后早期术后早期TVTV返流，右心功能严重衰竭，返流，右心功能严重衰竭，左心受累左心受累&右心右心EF:20EF:2025%25%、10101515 ECMOECMO支持时间支持时间8 8天、天、5 5天天&右心明显缩小：右心明显缩小：60 39mm60 39mm&左心功能明显好转，左心功能明显好转，EFEF：45 7045 70外科策略和原则外科策略和原则-总结和得到的启发总结和得到的启发JJRVRVRVRV功能好尽可能双心室矫治（功能好尽可能双心室矫治（功能好尽可能双心室矫治（功能好尽可能双心室矫治（ASDASDASDASD开窗）开窗）开窗）开窗）JJ新生儿期后可增加新生儿期后可增加新生儿期后可增加新生儿期后可增加GlennGlennGlennGlenn手术保证右心有效减压手术保证右心有效减压手术保证右心有效减压手术保证右心有效减压( ( ( (一个半心室）一个半心室）一个半心室）一个半心室）JJ急诊新生儿急诊新生儿急诊新生儿急诊新生儿StarnesStarnesStarnesStarnes或改良矫治是另一种选择（降低死亡率）或改良矫治是另一种选择（降低死亡率）或改良矫治是另一种选择（降低死亡率）或改良矫治是另一种选择（降低死亡率）JJ双心室和一个半心室矫治，双心室和一个半心室矫治，双心室和一个半心室矫治，双心室和一个半心室矫治，RVOTSRVOTSRVOTSRVOTS和和和和PSPSPSPS必须解除必须解除必须解除必须解除JJ对于严重右心衰竭影响左心的患者，可短期对于严重右心衰竭影响左心的患者，可短期对于严重右心衰竭影响左心的患者，可短期对于严重右心衰竭影响左心的患者，可短期ECMOECMOECMOECMO支持，待左心支持，待左心支持，待左心支持，待左心功能恢复后可逐渐脱离功能恢复后可逐渐脱离功能恢复后可逐渐脱离功能恢复后可逐渐脱离JJ晚期患者可考虑心脏移植晚期患者可考虑心脏移植晚期患者可考虑心脏移植晚期患者可考虑心脏移植