弯刀综合征14例外科治疗结果分析

Surgical outcomes of 14 children with scimitar syndrome

目的 探讨小儿弯刀综合征的诊断、外科治疗方法及治疗后的近、中期结果。方法 回顾性分析2010年1月到2017年12月手术治疗的14例弯刀综合征的临床资料。其中,男7例,女7例;年龄(18.23±28.55)个月,范围在23 d至9岁。合并其他心血管畸形10例,中位心8例,右肺发育不良7例,有侧支循环8例,合并肺动脉高压10例。用SPSS 16.0软件的寿命表(Life Table)做出本组患儿术后生存曲线,评估其近、中期生存状况。结果 9例异常肺静脉(scimitar vein,SV)从下腔静脉切下,端-侧吻合于左心房;SV分成2支2例,1例采用2支SV连同部分下腔静脉壁切下,另1例2支SV从下腔静脉从根部切下侧-侧吻合形成单根SV,均吻合于左心房;SV离左心房距离过大3例,2例将SV吻合在右心房右侧壁,再重建心房间隔,1例SV与自体心包卷管道连接再吻合于左心房。其他心血管畸形同期手术矫治。8例有侧支循环,3例心导管检查时被封堵,5例手术时切断缝合。本组14例手术死亡2例(14.29%),另有1例于术后1年2个月因肺炎、呼吸衰竭死亡。本组术后1、3、5年累积生存率分别为86%、73%和73%。术后出院时有1例移植的SV开口狭窄,血流速度为1.9 m/s;有3例随访期间发现移植SV狭窄,血流速度超过1.6 m/s。结论 弯刀综合征可合并其他心血管畸形、中位心、右肺发育不良等。一般将SV与左心房吻合;SV离左心房距离大或多条SV时,可采用个性化办法将SV吻合于左心房。侧支循环心导管检查时可封堵,或术中切断缝合。

Objective To summarize the diagnostic and surgical experience of children with scimitar syndrome and to explore the immediate/mid-term outcomes of these operations.Methods Retrospective analysis was performed for 14 clinical records of children with scimitar syndrome from January 2010 to December 2017.There were 7 boys and 7 girls with a mean age of(18.23±28.55 months)(23 days to 9 years).The complications included other cardiac abnormalities(n=10),mesocardia(n=8),right pulmonary hypoplasia(n=7)and collaterall circulation(n=8).Follow-ups were conducted for analyzing the immediate/mid-term outcomes of survival and stenosis of corrected scimitar vein(SV).And postoperative survival curves were plotted.Results SVs were dissected from inferior caval vein(IVC)and anastomosed to left artrium(n=9);SVs(2 SVs)were resected together with partial IVC wall and anastomosed to left artrium(n=1);SVs(2 SVs)were resected respectively and anastomosed side-to-side and then anastomosed to left artrium(n=1);SVs were anastomosed to right atrium and interatrium septum be rebuilt(n=2)and SV was connected to artificial conduit and then connected to left atrium(n=1)owing to that SV was too far to left atrium.In the same term of operation,complicated cardiac abnormality was cured.And 2/14 cases died immediately postoperatively with a mortality rate of 14.29%;1/12 survivors died of pneumonia and respiratory failure at 14 months postoperatively and 1/3/5-year postoperative cumulative survival rates were 86%,73%and 73%respectively.One corrected SV of 12 survivors was stenosed during discharge and 3 corrected SVs of 11 survivors became stenotic during follow-ups.Conclusions Scimitar syndrome may be complicated with other cardiac abnormality,mesocardia and pulmonary hypoplasia,etc.Most SVs of scimitar syndrome may be cured by anastomosing SV to left artrium.SV should be anastomosed to right atrium instead of left atrium and interatrium septum.Collateral circulation of scimitar syndrome may be plugged during cardiac interventions and even resected intraoperatively.