CAJ | 학술논문

目的研究功能性单心室手术矫治圆锥动脉干畸形合并二尖瓣骑跨(C型)的中远期疗效.方法 1989～2008年天津胸科医院单心室手术治疗7例圆锥动脉干畸形合并二尖瓣骑跨(C型),圆锥动脉干畸形包括大动脉转位,校正型大动脉转位,右室双出口.其他合并畸形包括圆锥部室间隔缺损,左室流出道狭窄等.完成双向格林手术1例,Fontan手术6例,全腔肺血管吻合5例,心房内隧道连接1例.结果术后随访5年1个月至13年.心脏超声检查发现中度二尖瓣关闭不全和三尖瓣关闭不全各1例.心导管检查发现轻度限制性室间隔缺损合并体肺动脉侧枝1例并栓堵侧枝,伞堵分流孔4例,4例术后需长期服药维护心功能,1例阵发性室上性心动过速需药物控制.NYHAⅠ级5例,Ⅱ级2例.结论若双心室修复无法保证圆锥动脉干畸型合并二尖瓣骑跨(C型)术后二尖瓣功能和其他畸形的彻底矫正,采用单心室手术既可避免双心室修复前姑息手术和相关并发症,还可简化手术避免复杂心脏畸形手术风险,中远期疗效较好.
목적 연구공능성단심실수술교치원추동맥간기형합병이첨판기과(C형)적중원기료효.방법 1989～2008년천진흉과의원단심실수술치료7례원추동맥간기형합병이첨판기과(C형),원추동맥간기형포괄대동맥전위,교정형대동맥전위,우실쌍출구.기타합병기형포괄원추부실간격결손,좌실류출도협착등.완성쌍향격림수술1례,Fontan수술6례,전강폐혈관문합5례,심방내수도련접1례.결과 술후수방5년1개월지13년.심장초성검사발현중도이첨판관폐불전화삼첨판관폐불전각1례.심도관검사발현경도한제성실간격결손합병체폐동맥측지1례병전도측지,산도분류공4례,4례술후수장기복약유호심공능,1례진발성실상성심동과속수약물공제.NYHAⅠ급5례,Ⅱ급2례.결론 약쌍심실수복무법보증원추동맥간기형합병이첨판기과(C형)술후이첨판공능화기타기형적철저교정,채용단심실수술기가피면쌍심실수복전고식수술화상관병발증,환가간화수술피면복잡심장기형수술풍험,중원기료효교호.
Objective To evaluate the functional outcome of univentricular repair for conotruncal anomalies associated with type C straddling mitral valve (SMV).Methods From 1989 to 2008,univentricular repair was performed on 7 patients with conotruncal anomalies associated with type C SMV.Conotruncal anomalies included transposition of great artery,corrected transposition of great artery and double outlet of right ventricle.The other cardiac malformations included conoventricular septal defect,left ventricular outflow tract obstruction,etc.Bidirection Gleen was performed on 1 case.Fontan was performed on 6 cases,including extracardiac conduit connection in 5 cases and lateral caval tunnel in 1 case.Results During the follow-up period ranging from 5 1/12 to 13 years,Ⅰ and Ⅱ degree of NYHA class were observed in 5 and 2 cases,respectively.Echocardiography revealed postoperative moderate mitral regurgitation and tricuspid regurgitation in 1 case.Respectively postoperative angiocardiography revealed mild restrictive VSD and systemic-pulmonary collaterial branches in 1 case.Coil embolization or Amplatzer fenestration closure was performed in 1 and 4 cases,respectively.Postoperative pharmacological therapy for maintaining cardiac function was required in 4 cases,including 1 case with paroxysmal supraventricular tachycardia.Conclusions For type C SMV associated with conotruncal anomalies,if successful repair for mitral valve malformation and other complex intracardiac defects could not be achieved,functional univentricular repair should be performed early with better midterm and long term outcome to avoid palliative operation and its related complications before biventricular repair,and to avoid operative risk of biventricular repair for complex intracardiac defects.