Ebstein畸形的外科治疗讲解材料

EbsteinEbstein畸形的外科治疗讲解材料畸形的外科治疗讲解材料 Ebstein畸形的外科治疗策略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Wilhelm Ebstein1866年首先描述形态Helen Taussig1950年描述临床特点解剖学特点1 Displacement of the septal and posterior leaflets of the TVtoward theapex of the RV 2 Although the anterior leaflet is attachedat theappropriate level of the tricuspid annulus it islarger thannormal andmay havemultiple chordalattachments to the ventricularwall 3 The segment of the RV from the level of thetrue tricuspid annulus to the levelof attachmentof the septaland posterior leafletsis unusuallythin anddysplastic The tricuspid annulus and the RAare extremelydilated 4 The cavityof the functional RVis reduced in size usually lacksan inletchamber and hasa smalltrabecular ponent 临床分型 分级 type A the volumeof thetrue RVis adequate type Bthere isa largeatrialized ponentof theRV but the anterior leafletmoves freely type C the anteriorleaflet isseverely restrictedin itsmovement andmay causesignficant obstructionof theRVOT type D there isalmost pleteatrialization of the ventriclewith theexception ofa smallinfundibular ponent The onlymunication between the atrializedventricle andthe infundibulumis throughthe anteroseptalmissure of the tricuspid valve 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11级 1 5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 22级61 NYHA3 44级21 病人死亡死亡病人有一项或多项特点1 NYHA3 44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 三尖瓣成形技术1 Danielson修复Ebstein畸形的治 疗2 改良Carpentier修复Ebstein畸形的外科治疗 3 改良Devega技 术runing bothends of the pledgettedsuture in and outalong the annulus separatingthe atrializedfrom the functional rightventricle from A to B the anteriorleaflet is not largeor ifthe posteriorleaflet iswell developedor ifboth the anterior andposterior leafletsare functionalbut dysplasticThe play itwhere itlies approach involveslimited plicationof the tricuspid valve Points Aand Bare approximatedwith1or2mattress suturesat the levelof the nativevalve not to the levelof thetrue tricuspidannulus This resultsin approximatingthe apicalaspects of the septaland anteriorleaflets effectively creatinga bicuspidvalve 4 前瓣单叶修复Ebstein畸形的外科治疗重症Ebste in畸形的定义 目前不明确 参考标准Predictors ofDeath inneonates withEbstein s Anomalycardiothoracic rationgreater than0 85 100 fatal Echocardiography scoregrade4 4 100 fatal Echocardiography scoregrade and cyanosis 100 fatal Severe tricuspid regurgitation mostly fatal Echocardiography scoregrade 45 fatal ininfancy Knott Craig CJet al Ann ThoracSurgxx 76 1786新生儿Ebstein畸形的治疗 Starnes矫治 J ThoracCardiovasc Surg1991 101 1082 7 5consecutive newborninfants Age 1 9days Weight 3 6 1 8kg MeanPH 7 2 0 05Mean oxygentension 29 6 2 3mmHg Meancardiothoracic ration 0 81 0 02ECHO severe tricuspidregurgitation functional pulmonary atresiain allpatients Allpatients wereresuscitated withintubation andmechanical ventilation acidosis wascorrected and therapywith PGE1 Preoperative echoassessment patientNo 12345RV dysplasia 00 tethered anteriorleaflet00 0 Echo scoreratio1 30 90 80 61 01severe TR functionalpulmonaryatresia Cardiac catheterizationassessment inone neonates Operative technique The tricuspid orifice wasclosed with autologous pericardium The coronarysinus beneaththe patchto reducethe riskof AVblock An ASDwas createdto ensuremixing at the atriallevel The rightatrium wasreduced insize byremoving asegmentof the right atrial freewall A A P shuntwas establishedwitha4mm Gore Tex vessel Results Noperioperative andlate deaths No postoperativearrhythmias Mechanical ventilationtime10 2 0 3days Po2 42 2 0 9mmHg SO2 83 2 1 9 Follow up Onereceived aGlenn operationafter11mo Two receivedFontan proceduresat23and22mo ofage 双心室矫治 Knott Craig CJ Repair of Ebstein s anomalyin thesymptomatic neonate an evolutionof techniquewith7 year follow up Ann ThoracSurgxx 73 1786 93 8symptomatic patients6neonates 2 19d 2 8 3 2kg 1young infant 2mo 3 8kg had undergonea starnesoperation elsewhere1infant 4mo 6 4kg 新生儿Ebstein畸形的治疗 Preopera tive assessment Severe 4 4 TR waspresent in all except1 Starnes operation Cardiothoracic ratioexceeded0 85in allpatients Echocardiography severityscores were 1 5in6 grade4 4 and1 3in1 grade3 4 3patients hadanatomical PA2had functionalPA新生儿Ebstein畸形的治疗 Operative techniqueRepair consistedof TVrepair Reduction atrioplasty Relief of RVOTobstruction Partialclosure ofASD Correction of all associated cardiac defects新生儿Ebstein畸形的治疗 Tricuspid valverepair 3had Danielson type repairs 3had DeVega type repairs and2had plexrepairs 1 modified Danielsontechnique placing a pledgettedsuture atthe A P missureand bringingthis downto theCS thus creatinga double orifice valve The lateralorifice containingthe atrialized RV which beclosed byplicating itvertically If thelarge anteriorleaflet doesnot coaptwell withthe ventricular septum a pledgettedsuture fromtheanteriorpapillary muscleto theventricularseptummay beused tocorrect this新生儿Ebstein畸形的治疗2 DeVega type annuloplasty theanteriorleaflet isnot largeor ifthe posteriorleafletiswell developedor ifboth theanterior andposteriorleafletsare functionalbut dysplastic runing bothends of the pledgettedsuture in and outalong theannulus separatingthe atrializedfromthe functional rightventricle from A to B 新生儿Ebstein畸形的治疗In themore severeforms ofEA in the neonate1 The orificeof theTV istoward theapex oftheRV 2 The missurebetweentheanterior andseptal leafletsmay beimperforate orpatent onlythrough smallfenestrations 3 The posteriorleaflet maybe reasonablywell developedand mobile 新生儿Ebstein畸形的治疗Detaching theentire anterior andposteriorleaflets fromtheannulusFreeing theleaflets fromtheir muscularizedattachments andreducing theannulus insize posteriorlyReattaching theleaflets tothe smallerannulus notonly correctsthe defectbut alsoeffectively changesthe orientationoftheTV backtotheRVOT andthe functionalRV Fenestrating theA S missureand leafletprevents tricuspidstenosis CorrectionofallassociatedcardiacdefectsPA PS orRVOTS RVOT patchorasmall homograftor othervalved conduitVSD more plex Unloading theRV FenestratedASD closureAdding thehemi Fontan connection in olderpatients Reductionatrioplasty Open rightpleural cavityand leavea drainin theperitoneal cavity新生儿Ebstein畸形的治疗 Results One patientdied inhospital no late deaths All arein sinusrhythm andin functionalclass I 4patients hadtrace tomild TRand2had mildto moderateregurgitation 外科矫治新观点 Sunil P Malhotra MD Selective RightVentricular Unloadingand NovelTechnical Conceptsin Ebstein s Anomalys San Francisco CA Jan26 28 xx New conecpts Using ofvalve reconstructivetechniques thatdiffer substantiallyfrom thoseintheliterature 1A play itwhere itlies approach tothetricuspid valve inwhich thereconstruction isperformed atthe functionalorifice insteadof movingthe valvetotheanatomic tricuspidannulus 2Avoidance ofdetachment andreimplantation ofvalve leaflets and3A limited plication performedonly atthe levelofthedisplaced valverather thanplete plicationoftheentire atrializedRV New conecpts Depending specificphysiologic andanatomic criteriafor selectiveuse ofthe BDG in conjunctionwith repairofEbstein s anomaly Patient Characteristics93 12 08 1257consecutive patientsoutside ofthe neonatalperiod Thediagnosis ofsevere Ebstein s anomalyofthetricuspidvalvewas establishedby echocardiographyinallpatients Echocardiography was used tocharacterize thedegree ofapical displacement ofthetricuspidannulus the severityand natureof TR andthedegree ofmobility oftheanteriorleaflet TR wasclassified ona scaleof1to4 1 trace 2 mild 3 moderate and4 severe Echocard iography alsowasusedto assessright and left ventricularfunction andto identifyany atriallevel shunts Patient CharacteristicsAge 7months to40 4years exerciseintolerance in40cyanosis in26RV failurein18atrial dysrhythmiasin8TR wasmoderate orsevere in50patients 87 7 Approaches tothe Tricuspid Valve1The detrimentaleffects ofa verylarge tricuspidannulus ApproachestotheTricuspidValve2The goalof plicationoftheatrializedRVThe play itwhere itlies approach involveslimitedplicationofthetricuspidvalve Points Aand Bare approximatedwith1or2mattress suturesatthelevelofthe nativevalve not tothelevelofthetrue tricuspidannulus This resultsin approximatingthe apicalaspects oftheseptaland anteriorleaflets effectively creatinga bicuspidvalve 3Selective useoftheBDG using theBDGintwo separateand independentcircumstances The firstis physiologic Cyanosis at rest isa markerfor an inadequate RV pump If thepatient isfully saturatedatrestbut beescyanotic withexercise this isa relativemarker of aninadequateRVpump and we will havea lowthreshold forplacing a BDG Typically we willseparate thepatient from cardiopulmonary bypassafter valverepair andmonitor rightandleft atrial pressure If therightatrialpressure exceeds1 5times theleftatrialpressure underthese relativelyunstressed conditionsofanopen chestinananesthetized patient we willperform a BDG If thepatient presentswith anintact atrialseptum oran atrialseptal defectwith left to right shunting aBDG isnotperformed The secondcircumstance forplacingaBDGisanatomic andrelates tothe ultimatesize ofthefunctionaltricuspidannulusafter repair If itis necessaryto makethefunctionaltricuspidorificesubstantially lessthan2 5cm ina70 kg patient to achievea petent valve wewillassess inflowvelocity acrossthetricuspidafter separationfromcardiopulmonarybypass usingtransesophageal echocardiography If obstructionis demonstrated aBDGis placed We acknowledgethat manyofthemaneuvers usedto makea regurgitantvalve petentinvolve reducingthe valveopening This optionfor BDGuse freesus toaggressively reducethefunctionalvalve orificeas muchas necessaryto achievea stable petentvalverepair Conitant ProceduresPerformed atInitial Ebstein s AnomalyRepair ProceduresNo Electrophysiologic procedures8Ablation ofaessory pathway2Maze proceduresBilatera l2With pacemaker1Right sided3With pacemaker1Pacemaker alone1Partial anomalouspulmonary veinrepair1Pulmonary valvereplacement1ReliefofRV outflowtract obstruction2Supravalvar pulmonarystenosis repair1Results Noearly orlatedeathsourred Early reoperationwas requiredin2patients 1patient requiredpacemaker placementfo