�306��第二军医大学学报�2010年3月第31卷第3期http://www.ajsmmu.cnAcademicJournalofSecondMilitaryMedicalUniversity,Mar.2010,Vo.l31,No.3DOI:10.3724/SP.J.1008.2010.00000�研究快报�无顶冠状静脉窦综合征的诊断与外科治疗薛�清,韩�林,张冠鑫,陆方林,邹良建,郎希龙,徐激斌,徐志云*第二军医大学长海医院胸心外科,上海200433��[摘要]�目的�探讨并总结无顶冠状静脉窦综合征(UCSS)的临床表现和外科处理方法。方法�1999年1月至2009年6月我科共收治9例UCSS患者,其中a型3例、b型1例、!a型1例、∀a型3例、∀b型1例。术前经超声心动图检查明确诊断6例,术中探查明确诊断3例。合并永存左上腔静脉(PLSVC)共7例,其中PLSVC直接汇入左房(a型)3例,采取房间隔重建术1例,心内隧道术2例;PLSVC汇入冠状静脉窦后同时开口于左右房(!a型)1例,开口于左房(∀a型)3例,均处理冠状静脉窦左房开口。合并其他心内畸形或继发性改变同期手术矫治。结果�术后早期2例出现并发症,余7例恢复顺利。全组均痊愈出院,1例失访,8例随访2个月至5年,无死亡和晚期并发症发生。结论�UCSS是一种罕见的先天性心脏病,术前诊断有一定困难,术中须仔细探查,可根据PLSVC连接心房的方式、冠状静脉窦异常开口的位置、合并其他心内畸形或继发性改变的特点选择手术方法,如能达到生理性矫治则预后良好。��[关键词]�无顶冠状静脉窦综合征;外科治疗;永存左上腔静脉��[中图分类号]�R654.2���[文献标志码]�A���[文章编号]�0258-879X(2010)03-0306-04DiagnosisandsurgicaltreatmentofunroofedcoronarysinussyndromeXUEQing,HANLin,ZHANGGuan-xin,LUFang-lin,ZOULiang-jian,LANGXi-long,XUJi-bin,XUZhi-yun*DepartmentofCardiothoracicSurgery,ChanghaiHospita,lSecondMilitaryMedicalUniversity,Shanghai200433,China[收稿日期]�2009-12-07����[接受日期]�2010-01-26[作者简介]�薛�清,硕士生.E-mai:lxq9911310@yahoo.com.cn*通讯作者(Correspondingauthor).Te:l021-81873417,E-mai:lzhiyunx@hotmai.lcom��[Abstract]�Objective�Tosummarizetheclinicalmanifestationsandsurgicaltreatmentofunroofedcoronarysinussyndrome(UCSS).Methods�NinepatientsunderwentsurgicalrepairforUCSSfromJan.1999toJun.2009inourhospita,landthepatientsincluded3withtypea,1withtypeb,1withtype!a,3withtype∀a,and1withtype∀bUCSS.ThediagnosisofUCSSwasmadebyechocardiographyin6patientsandbythesurgicalexplorationin3patients.Persistentleftsuperiorvenacava(PLSVC)wasfoundin7cases;3caseswithPLSVCdirectlydrainingintotheleftatrium(a)werereconstructedbytheintra-atrialroutestoguidePLSVCtotherightatrium,and4caseswithPLSVCdrainingintothecoronarysinusandcoronarysinusorificeopeningtotheleftatriumandrightatrium(1case,!a)andleftatrium(3cases,∀a)weredealtwiththeabnormalcoronarysinusorifice.Otherassociatedcardiaclesionswerecorrectedconcomitantly.Results�Twocaseshadearlycomplicationsandtheother7caseshadsmoothrecoveryafteroperation;allthepatientswerecuredanddischarged.Eightcaseswerefollowedupfor2monthsto5yearsandtherewasnodeathorlatecomplications.Conclusion�UCSSisararecongenitalcardiacanomalyandisdifficulttodiagnosepreoperatively;socarefulexplorationshouldbedoneduringoperation.DifferentsurgicalapproachesshouldbechosenaccordingtothetypesofcommunicationbetweenPLSVCandcardiacatrium,thepositionsofabnormalsinusostium,andothercardiaclesions.Satisfactoryprognosiscanbeobtainedifphysiologicalcorrectionisachieved.��[Keywords]�unroofedcoronarysinussyndrome;surgicalprocedures;persistentleftsuperiorvenacava[AcadJSecMilMedUniv,2010,31(3):306-309]��无顶冠状静脉窦综合征(UCSS)又称冠状静脉窦间隔缺损,是由于胚胎发育时期左房静脉皱襞即冠状静脉窦间隔部分或完全缺损,使冠状静脉窦与左房直接交通,而形成的一组罕见的先天性心...
