Ebstein畸形的外科治疗医学幻灯片.ppt

Ebstein畸形的外科治疗策略 1 医药交流PPT 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 2 医药交流PPT 解剖学特点 DisplacementoftheseptalandposteriorleafletsoftheTVtowardtheapexoftheRV Althoughtheanteriorleafletisattachedattheappropriatelevelofthetricuspidannulus itislargerthannormalandmayhavemultiplechordalattachmentstotheventricularwall 3 医药交流PPT 3 ThesegmentoftheRVfromthelevelofthetruetricuspidannulustothelevelofattachmentoftheseptalandposteriorleafletsisunusuallythinanddysplastic ThetricuspidannulusandtheRAareextremelydilated 4 ThecavityofthefunctionalRVisreducedinsize usuallylacksaninletchamber andhasasmalltrabecularcomponent 4 医药交流PPT 5 Theinfundibulumisoftenobstructedbytheredundanttissueoftheanteriorleafletaswellasbythechordalattachmentsoftheanteriorleaflettotheinfundibulum 5 医药交流PPT 临床分型 分级 typeA thevolumeofthetrueRVisadequate typeB thereisalargeatrializedcomponentoftheRV buttheanteriorleafletmovesfreely typeC theanteriorleafletisseverelyrestrictedinitsmovementandmaycausesignficantobstructionoftheRVOT typeD thereisalmostcompleteatrializationoftheventriclewiththeexceptionofasmallinfundibularcomponent Theonlycommunicationbetweentheatrializedventricleandtheinfundibulumisthroughtheanteroseptalcommissureofthetricuspidvalve 6 医药交流PPT 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 1 5 7 医药交流PPT 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 8 医药交流PPT 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 9 医药交流PPT 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 10 医药交流PPT 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 11 医药交流PPT 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 12 医药交流PPT 三尖瓣成形技术 1 Danielson修复 Ebstein畸形的治疗 13 医药交流PPT 2 改良Carpentier修复 Ebstein畸形的外科治疗 14 医药交流PPT 3 改良Devega技术 runingbothendsofthepledgettedsutureinandoutalongtheannulusseparatingtheatrializedfromthefunctionalrightventriclefrom A to B theanteriorleafletisnotlargeoriftheposteriorleafletiswelldevelopedorifboththeanteriorandposteriorleafletsarefunctionalbutdysplastic The playitwhereitlies approachinvolveslimitedplicationofthetricuspidvalve PointsAandBareapproximatedwith1or2mattresssuturesatthelevelofthenativevalve nottothelevelofthetruetricuspidannulus Thisresultsinapproximatingtheapicalaspectsoftheseptalandanteriorleaflets effectivelycreatingabicuspidvalve 15 医药交流PPT 4 前瓣单叶修复 Ebstein畸形的外科治疗 16 医药交流PPT 重症Ebstein畸形的定义 目前不明确参考标准PredictorsofDeathinneonateswithEbstein sAnomalycardiothoracicrationgreaterthan0 85 100 fatal Echocardiographyscoregrade4 4 100 fatal Echocardiographyscoregrade andcyanosis 100 fatal Severetricuspidregurgitation mostlyfatal Echocardiographyscoregrade 45 fatalininfancy Knott CraigCJetal AnnThoracSurg2002 76 1786 17 医药交流PPT 新生儿Ebstein畸形的治疗 Starnes矫治 JThoracCardiovascSurg1991 101 1082 7 5consecutivenewborninfantsAge 1 9days Weight 3 6 1 8kgMeanPH 7 2 0 05Meanoxygentension 29 6 2 3mmHgMeancardiothoracicration 0 81 0 02ECHO severetricuspidregurgitationfunctionalpulmonaryatresiainallpatientsAllpatientswereresuscitatedwithintubationandmechanicalventilation acidosiswascorrected andtherapywithPGE1 18 医药交流PPT PreoperativeechoassessmentpatientNo 12345RVdysplasia 00 tetheredanteriorleaflet00 0 Echoscoreratio1 30 90 80 61 01severeTR functionalpulmonaryatresia 19 医药交流PPT Cardiaccatheterizationassessmentinoneneonates 20 医药交流PPT Operativetechnique Thetricuspidorificewasclosedwithautologouspericardium ThecoronarysinusbeneaththepatchtoreducetheriskofAVblock AnASDwascreatedtoensuremixingattheatriallevel 21 医药交流PPT Therightatriumwasreducedinsizebyremovingasegmentoftherightatrialfreewall AA Pshuntwasestablishedwitha4mmGore Texvessel 22 医药交流PPT Results Noperioperativeandlatedeaths Nopostoperativearrhythmias Mechanicalventilationtime10 2 0 3days Po2 42 2 0 9mmHg SO2 83 2 1 9 23 医药交流PPT Follow up OnereceivedaGlennoperationafter11mo TworeceivedFontanproceduresat23and22moofage 24 医药交流PPT 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 25 医药交流PPT Preoperativeassessment Severe 4 4 TRwaspresentinallexcept1 Starnesoperation Cardiothoracicratioexceeded0 85inallpatientsEchocardiographyseverityscoreswere 1 5in6 grade4 4 and1 3in1 grade3 4 3patientshadanatomicalPA2hadfunctionalPA 新生儿Ebstein畸形的治疗 26 医药交流PPT Operativetechnique RepairconsistedofTVrepairReductionatrioplastyReliefofRVOTobstructionPartialclosureofASDCorrectionofallassociatedcardiacdefects 新生儿Ebstein畸形的治疗 27 医药交流PPT 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 28 医药交流PPT 2 DeVega typeannuloplasty theanteriorleafletisnotlargeoriftheposteriorleafletiswelldevelopedorifboththeanteriorandposteriorleafletsarefunctionalbutdysplastic runingbothendsofthepledgettedsutureinandoutalongtheannulusseparatingtheatrializedfromthefunctionalrightventriclefrom A to B 新生儿Ebstein畸形的治疗 29 医药交流PPT InthemoresevereformsofEAintheneonate 1 TheorificeoftheTVistowardtheapexoftheRV 2 Thecommissurebetweentheanteriorandseptalleafletsmaybeimperforateorpatentonlythroughsmallfenestrations 3 Theposteriorleafletmaybereasonablywelldevelopedandmobile 新生儿Ebstein畸形的治疗 30 医药交流PPT Detachingtheentireanteriorandposteriorleafletsfromtheannulus Freeingtheleafletsfromtheirmuscularizedattachmentsandreducingtheannulusinsizeposteriorly ReattachingtheleafletstothesmallerannulusnotonlycorrectsthedefectbutalsoeffectivelychangestheorientationoftheTVbacktotheRVOTandthefunctionalRV FenestratingtheA Scommissureandleafletpreventstricuspidstenosis 31 医药交流PPT CorrectionofallassociatedcardiacdefectsPA PSorRVOTS RVOTpatchorasmallhomograftorothervalvedconduitVSD morecomplexUnloadingtheRVFenestratedASDclosureAddingthehemi Fontanconnection inolderpatients ReductionatrioplastyOpenrightpleuralcavityandleaveadrainintheperitonealcavity 新生儿Ebstein畸形的治疗 32 医药交流PPT Results OnepatientdiedinhospitalnolatedeathsAllareinsinusrhythmandinfunctionalclassI4patientshadtracetomildTRand2hadmildtomoderateregurgitation 33 医药交流PPT 外科矫治新观点 SunilP MalhotraMD SelectiveRightVentricularUnloadingandNovelTechnicalConceptsinEbstein sAnomalys SanFrancisco CA Jan26 28 2009 Newconecpts Usingofvalvereconstructivetechniquesthatdiffersubstantiallyfromthoseintheliterature 1A playitwhereitlies approachtothetricuspidvalveinwhichthereconstructionisperformedatthefunctionalorificeinsteadofmovingthevalvetotheanatomictricuspidannulus 2Avoidanceofdetachmentandreimplantationofvalveleaflets and3AlimitedplicationperformedonlyatthelevelofthedisplacedvalveratherthancompleteplicationoftheentireatrializedRV 34 医药交流PPT Newconecpts DependingspecificphysiologicandanatomiccriteriaforselectiveuseoftheBDGinconjunctionwithrepairofEbstein sanomaly 35 医药交流PPT PatientCharacteristics 93 12 08 1257consecutivepatientsoutsideoftheneonatalperiodThediagnosisofsevereEbstein sanomalyofthetricuspidvalvewasestablishedbyechocardiographyinallpatients Echocardiographywasusedtocharacterizethedegreeofapicaldisplacementofthetricuspidannulus theseverityandnatureofTR andthedegreeofmobilityoftheanteriorleaflet TRwasclassifiedonascaleof1to4 1 trace 2 mild 3 moderate and4 severe Echocardiographyalsowasusedtoassessrightandleftventricularfunctionandtoidentifyanyatriallevelshunts 36 医药交流PPT PatientCharacteristics Age 7monthsto40 4yearsexerciseintolerancein40cyanosisin26RVfailurein18atrialdysrhythmiasin8TRwasmoderateorseverein50patients 87 7 37 医药交流PPT ApproachestotheTricuspidValve 1Thedetrimentaleffectsofaverylargetricuspidannulus 38 医药交流PPT ApproachestotheTricuspidValve 2ThegoalofplicationoftheatrializedRV The playitwhereitlies approachinvolveslimitedplicationofthetricuspidvalve PointsAandBareapproximatedwith1or2mattresssuturesatthelevelofthenativevalve nottothelevelofthetruetricuspidannulus Thisresultsinapproximatingtheapicalaspectsoftheseptalandanteriorleaflets effectivelycreatingabicuspidvalve 39 医药交流PPT 3SelectiveuseoftheBDG usingtheBDGintwoseparateandindependentcircumstances Thefirstisphysiologic CyanosisatrestisamarkerforaninadequateRVpump Ifthepatientisfullysaturatedatrestbutbecomescyanoticwithexercise thisisarelativemarkerofaninadequateRVpump andwewillhavealowthresholdforplacingaBDG Typically wewillseparatethepatientfromcardiopulmonarybypassaftervalverepairandmonitorrightandleftatrialpressure Iftherightatrialpressureexceeds1 5timestheleftatrialpressureundertheserelativelyunstressedconditionsofanopenchestinananesthetizedpatient wewillperformaBDG Ifthepatientpresentswithanintactatrialseptumoranatrialseptaldefectwithleft to rightshunting aBDGisnotperformed 40 医药交流PPT ThesecondcircumstanceforplacingaBDGisanatomicandrelatestotheultimatesizeofthefunctionaltricuspidannulusafterrepair Ifitisnecessarytomakethefunctionaltricuspidorificesubstantiallylessthan2 5cm ina70 kgpatient toachieveacompetentvalve wewillassessinflowvelocityacrossthetricuspidafterseparationfromcardiopulmonarybypassusingtransesophagealechocardiography Ifobstructionisdemonstrated aBDGisplaced Weacknowledgethatmanyofthemaneuversusedtomakearegurgitantvalvecompetentinvolvereducingthevalveopening ThisoptionforBDGusefreesustoaggressivelyreducethefunctionalvalveorificeasmuchasnecessarytoachieveastable competentvalverepair 41 医药交流PPT ConcomitantProceduresPerformedatInitialEbstein sAnomalyRepairProceduresNo Electrophysiologicprocedures8Ablationofaccessorypathway2MazeproceduresBilateral2Withpacemaker1Right sided3Withpacemaker1Pacemakeralone1Partialanomalouspulmonaryveinrepair1Pulmonaryvalvereplacement1ReliefofRVoutflowtractobstruction2Supravalvarpulmonarystenosisrepair1 42 医药交流PPT Results Noearlyorlatedeathsoccurred Earlyreoperationwasrequiredin2patients 1patientrequiredpacemakerplacementforatrioventricularnodalblocka