无顶冠状静脉窦综合征

无顶冠状静脉窦综合征又称冠状窦间隔缺损，是一种罕见的先天性心脏畸形。作者手术治疗９例，其中永存左上腔静脉（ＰＬＳＶＣ）直接汇入左心房（ＬＡ），即完全型冠状窦间隔缺损７例，ＰＬＳＶＣ汇入冠状静脉窦（ＣＳ）开口，于ＬＡ即终端部份型冠状窦间隔缺损１例，另１例为完全型肺静脉异位引流入ＣＳ伴中间部分型冠状窦间隔缺损。９例均合并其他心脏畸形。全组无手术死亡。术前术中诊断困难。较为可靠的术前诊断方法是心导管和超声心动图检查，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，更取决于ＰＬＳＶＣ是否存在和能否结扎。若不伴有ＰＬＳＶＣ或伴有ＰＬＳＶＣ能够结扎，则只需用补片修补房缺和关闭ＣＳ开口，或补片修补房缺时，将ＣＳ开口隔向ＬＡ侧。若伴有ＰＬＳＶＣ不能结扎，可采用建立左房内隧道或重建房间隔，将直接引流入ＬＡ或汇入ＣＳ的ＰＬＳＶＣ导流或折流向ＲＡ。手术死亡率主要取决于合并畸形的复杂性和严重程度。

Unroofedcoronarysinussyndrome

Unroofedcoronarysinussyndromeorcoronarysinusseptaldefectisararecongenitalcardiacanomaly.Weperformedcorrectiveoperationsin9patientswithsuchcondition.Ofthem,sevenpatientshadthecompletelyunroofedcoronarysinusandpersistentleftsuperiorvenacava(PLSVC)dircetlydrainingintotheleftatrium(LA).OnepatienthadPLSVCandthepartialyunroofedterminalportionofthecoronarysinus(CS),orsinusostiumopenintoLA.Anotherpatienthadtotalanomalouspulmonaryveinconnectionandthepartialyunroofedmid-portionofCSwithoutPLSVC.Theoperativemethodsoftheunroofedcoronarysinusincluded:ligatingPLSVCandrepairingostiumprimumorse-cundumatrialseptaldefectsin4cases;creatinganintra-atrialtunnelfromPLSVCtotherightatrium(RA)andrepairingtheatrialseptaldefectin2cases;reconstructingtheintra-atrialseptalwithapatchasabafletoguidePLSVCorsinusostiumrespectivelytowardsRAin2cases;enlargingthedefectonthecoronarysinusroofandrepairingtheatrialseptaldefectofthecoronarysinustypeinonecase.Nopatientdiedinthisgroup.Becauseunroofedcoronarysinussyndromehasatypicalclinicalmanifestationandusualycomplicatesvariedcongenitalanomalies,itspreoperativediagnosisisverydifficultinsometimesandmaydrawthereliablesupportfromechocardiographyandcatheterization.Theoperativepro-gramandmethodmustdependnotonlyuponitstypebutalsouponPLSVCpresenceorabsence,andcommunicationbetweenPLSVCandRSVC.