EbsteinEbstein畸形的外科治疗畸形的外科治疗策略策略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发生率发生率1:40,000-200,0001:40,000-200,000先天性心脏病中先天性心脏病中:<1%:<1%疾病谱宽：疾病谱宽：轻型无症状轻型无症状重症新生儿期死亡重症新生儿期死亡率极高率极高手术死亡率手术死亡率高高WilhelmEbstein1866年首先描述形态HelenTaussig1950年描述临床特点解剖学特点解剖学特点1.1.DisplacementoftheseptalandposteriorlDisplacementoftheseptalandposteriorleafletsoftheTVtowardtheapexoftheReafletsoftheTVtowardtheapexoftheRV.V.2.2.AlthoughtheanteriorleafletisattachedatAlthoughtheanteriorleafletisattachedattheappropriatelevelofthetricuspidannutheappropriatelevelofthetricuspidannulus,itislargerthannormalandmayhavelus,itislargerthannormalandmayhavemultiplechordalattachmentstotheventrimultiplechordalattachmentstotheventricularwall.cularwall.3.ThesegmentoftheRVfromthelevelofthet3.ThesegmentoftheRVfromthelevelofthetruetricuspidannulustothelevelofattachmruetricuspidannulustothelevelofattachmentoftheseptalandposteriorleafletsisunusentoftheseptalandposteriorleafletsisunusuallythinanddysplastic.Thetricuspidannuluallythinanddysplastic.ThetricuspidannulusandtheRAareextremelydilated.usandtheRAareextremelydilated.4.ThecavityofthefunctionalRVisreducedin4.ThecavityofthefunctionalRVisreducedinsize,usuallylacksaninletchamber,andhassize,usuallylacksaninletchamber,andhasasmalltrabecularcomponent.asmalltrabecularcomponent.5.5.TheinfundibulumisoftenobstructedbTheinfundibulumisoftenobstructedbytheredundanttissueoftheanteriorleytheredundanttissueoftheanteriorleafletaswellasbythechordalattachmeafletaswellasbythechordalattachmentsoftheanteriorleaflettotheinfundintsoftheanteriorleaflettotheinfundibulum.bulum.临床分型（分级）临床分型（分级）typeA:typeA:thevolumeofthetrueRVisadequate.thevolumeofthetrueRVisadequate.typeBtypeB：：thereisalargeatrializedcomponentofthereisalargeatrializedcomponentoftheRV,buttheanteriorleafletmovesfreely.theRV,buttheanteriorleafletmovesfreely.typeC:typeC:theanteriorleafletisseverelyrestrictedinitsmovementandtheanteriorleafletisseverelyrestrictedinitsmovementandmaycausesignficantobstructionoftheRVOT.maycausesignficantobstructionoftheRVOT.typeD:typeD:thereisalmostcompleteatrializationoftheventriclewiththethereisalmostcompleteatrializationoftheventriclewiththeexceptionofasmallinfundibularcomponent.Theonlycommunicationexceptionofasmallinfundibularcomponent.Theonlycommunicationbetweentheatrializedventricleandtheinfundibulumisthroughthebetweentheatrializedventricleandtheinfundibulumisthroughtheanteroseptalcommissureofthetricuspidvalve.anteroseptalcommissureofthetricuspidvalve.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//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11级：级：<<＝＝0.50.522级：级：0.6-1.00.6-1.033级：级：1.1-1.51.1-1.544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PAAPAA4.4.左室受压，呈“夹心饼”，功能受限左室受压，呈“夹心饼”，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临床表现：临床表现：容易疲劳，活...
