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1、无顶冠状静脉窦综合征'^无顶冠状静脉窦综合征梁继河刘维永,,刘建萍杨案学汪刚李军张军李敬邦——～————～摘要无顶冠静脉赛综台矩称冠状安间隔敞损,是种罕见的先天性心脏畸形=作者手术治疗9倒,其中永存左J二脏静脉(PLSVC)直接订入:心房(IA).即完全冠状窦间隔缺损7倒,PLSVC汇八冠状静脉赛(cs)开l1,—LA即终端部份嘲冠状宴间隔歃损1例,另l倒为完全型肺静脉异位引流^CS伴中间部分型冠状赛问隔缺损9倒均合并其他L-脏畸形.皂组无手术死亡术前术中诊断困难较为叮靠的术前诊断方法是心导管和超声心动图世查,但术中心外,心内探查至关重要.手术治疗方案虽取决其类型,更取决1:PI.SVC是否
2、存在和能否结扎.若佯有PISVC或伴有PLSVC能够结扎,则只需用补片修补房缺和关闭CS开u.或补片修补庸缺时.将CS开口隔向LA侧.若伴有PLSVC不能结扎,可采用建左房内隧道或重建房间隔,将直接引流AIA或汇凡('s的PISVC导流或折流向RA;手术死亡率主要取决于台并畸形的复杂性和严重程度.关键词心脏外科手术冠状血管畸形Unroofedeorollar~sinuss)ndromeLianghhe,LiuWeiyongi,￡"h'anping,etd}.DepartmentofCardio-thoracicSurgery—x"ngHospital,FourthMilitaryfvtedi
3、eal:~eiversity,Xi.710032AbstractUnrooledcoronarysJllussyndromeorcoro11~rysinussepialdefeetararecongenitalcardiacanomaly.Weperformedcorrectiveoperationsin9palienLSwithsuchcondition.Ofthem.seve11patielltshadthecomp[erelyU1]roofedcorollarysinusand呻rs[stentleftsuperiorvellacav8(PLSVC)dircetlydra】nlngin
4、totheIeft8tritlm(LA)OnepatienthadPI.SVCandthepartiallyunroofedtermillalportionofthecoronarysinus(CS).orsinusostiumopenintol_A.A11otherpatienthadtotalanotTRtlouspuhnonaryveinconnectionandthepartia】【YLlllro0fedmidportionofCSwithoutPLSVCTheoperativemethodsoftheunroofedcoronarysinusincluded:】igatingPl_
5、SVCandrepairingostiumprimumorseeundumatria】septaldefectsindcases;creatinganintraatria】tunnelfromPLSVCtotherightatrium(RA)andrepairingtheatrialseptaldefectin2cases;reconstructi11gtheintra—atrialseptalwithapatehasabaffletoguidePI.SVCorsinusosliumrespectivelytoward~RAin2casesenlargingthedefectonthecor
6、onarysinusroofandrepairingtheatrialsepla】defectofthecoronarysinustypeino…ase.NopatientdiedjnthisgroupBecauseunmofedcoronarysinussyndromehas8typicaIclinicalmanifestationandusuallycomplicatesvariedcongenitalanoma¨es,Itspreoperat【vediagnosisisverydifficult【nson1etimesandmaydrawthereliablesupportfrolrt
7、echocardiographyandcatheterizationThe0DeratireprogramandmethodmustdependnotonlyuponitstypebutalsouponPI.SVCpresenceorabsence.andcomillullicationbetw~e11PLsvCandRSVC.KeywordsHcartsurgeryCoronarYveNselan[_iii
