66例部分性房室管畸形的外科治疗

66例部分性房室管畸形的外科治疗
郑家豪薛松朱洪生殷荣黄日太
（上海交通大学医学院附属仁济医院心胸外科，上海200001）
摘要：目的总结部分性房室管畸形手术治疗的经验，以提高治疗效果。方法回顾性分析我院1984年1月～2007年12月经外科治疗部分性房室管畸形患者66例的临床资料，对二尖瓣大瓣裂的处理：单纯裂缺缝合52例，缝合加交界折叠缝缩8例，加小瓣成形1例，加缝置St.Jude软质人工成形环3例，人工机械瓣置换术2例；对原发孔型房间隔缺损的修补：采用涤纶补片修补12例，自体心包54例；采用Kirklin法将冠状静脉窦口隔入左心房5例，MeGoon法将冠状静脉窦口隔入右心房61例；同期处理其他合并畸形。结果术后早期死亡2例（3.03%），1例死于心律失常，另1例死于呼吸功能衰竭。术后发生Ⅲ°房室传导阻滞2例，均安装永久性心脏起搏器。术后随访52例（81.3%），随访时间5个月～22年，平均15年，心功能均有明显改善，尤其是术前心功能Ⅲ~Ⅳ级的19例患者，术后改善为Ⅰ~Ⅱ级。再次手术4例，其中1例经再次行二尖瓣置换术治愈；术后死亡3例，1例死于急性肾功能衰竭，2例死于低心排血量综合征。结论早期手术治疗可以保全房室瓣结构、功能，避免发生
肺动脉高压和降低死亡率。手术治疗的关键是消除二尖瓣关闭不全、修补原发孔型房间隔缺损和避免损伤传导组织，术后近、中期疗效良好；有残留中度以上二尖瓣反流者，远期效果不满意。
关键词:部分性房室管畸形；房间隔缺损；二尖瓣关闭不全；完全性房室传导阻滞
中图分类号：R654.2文献标识码：A文章编号：1007-4848（2009）02-0102-04
SurgicalTreatmentofPartialAtrioventricularCanalDefectin66CasesZHENGJia-hao,XUESong,ZHUHong-sheng,YINGRong,HUANGRi-tai.（DepartmentofCardiothoracicSurgery,RenjiHospital,ShanghaiJiaotongUniversity,SchoolofMedicine,Shanghai200001,P.R.China.E-mail:jasonzhengjh@163.com）Abstract:ObjectiveTosummarizetheexperiencesofsurgicaltreatmentforpartialatrioventricularcanaldefect.MethodsThedataof66patientsofsurgicaltreatmentforpartialatrioventricularcanaldefectfromJanuary1984toDecember2007wereanalyzedretrospectively.Thecleftofmitralvalvepresentedinallofthosepatients.Therewere52caseswithdirectsutureoncleft,8caseswithdirectsuturewithcommissurroplasty,1casewithposteriorleafletplasty,3caseswithdirectsutureSt.Juderingand2casesmitralvalvereplacement.TheostiumprimumatrialseptaldefectswererepairedwithpatchesofDacronin12casesandautologouspericardiumin54cases.Coronarysinuswassituatedontheleftatriumin5andostiumprimumatrialseptaldefectswererepairedinKirklin’sway;theothersinMeGoon'way.Meanwhileotherheartabnormalitiesweredone.ResultsThereweretwoearlydeaths（3.03%）,onepatientdiedofheartarrhythmiaandonepatientdiedofrespiratoryfailure.ComplicationsoftotalAVblockwasin2cases.Bothofthemwerereplantedwithpacemakers.52caseswerefollowedup,followuptimewas5monthsto22years（meanfollowup15years）.Allpatientshadbetterlife.Fourpatientshavebeenreoperatedfordifferentreasonspostprimaryoperation.Onehadgoodresultafterremitralvalvereplacement.Onecasediedofacuterenalfailureandtheothertwodiedoflowcardiacoutputsyndrome.ConclusionsEarlyoperationisdefinitelyrecommendedwhenthediagnosisisconfirmed.Becausethestructureorfunctionofmitralvalveissaved,pulmonaryhypertensionisavoidedandthemortalityislowerinthefuture.Thekeypointsofoperationaretorectifythemitralinsufficiency,repairostiumprimumatrialseptaldefectsandavoidatrioventricularblock.Thepatientsofmildregurgitationofmitralvalvehavegoodresults.Providedinthosehavemorethanmiddleregurgitationofmitralvalvethentheirlongtermresultsarepoor.
Keywords:Partialatrioventricularcanaldefect;Atrialseptaldefect;Mitralvalveinsufficiency;Completeatrioventricularblock