弯刀综合征术后肺静脉狭窄1例报道并文献复习  

Postoperative pulmonary vein stenosis in scimitar syndrome:1 case report and literature survey

作　　者：宋海龙[1] 杨仕海[1] 陶曙光[1] 谷疆蓉[1] 韩剑刚[1] 温林林[1] 金立臣[1] 姚俊平[1] 韩喆[1] SONG Hailong;YANG Shihai;TAO Shuguang;GU Jiangrong;HAN Jiangang;WEN Linlin;JIN Lichen;YAO Junping;HAN Zhe(Department of Cardiac Surgery,Children＇s Hospital of Hebei Province,Shifiazhuang 050031,China)

机构地区：[1]河北省儿童医院心脏外科,050031

出　　处：《心肺血管病杂志》2018年第8期774-778,共5页Journal of Cardiovascular and Pulmonary Diseases

基　　金：河北省2015年度医学科学研究重点课题计划(20150564)

摘　　要：目的:复习肺静脉异位引流术后肺静脉狭窄的相关文献,了解这类患儿的临床特点。方法:回顾分析我院心外科治疗的1例弯刀综合征术后肺静脉狭窄并两次行肺静脉狭窄矫治术患儿的临床资料,并以"肺静脉异位引流术后肺静脉狭窄"为检索词,在万方数据库和中国全文期刊数据库进行检索,以"toal pulmonary venous connection"为检索词在pubmed全文数据库中进行检索。结果:此例弯刀综合征术后7个月发现肺静脉狭窄,第一次行肺静脉狭窄矫治术,术中发现肺静脉切口处远心端内膜增厚伴狭窄,切除增厚的内膜,扩大肺静脉切口,并采用SATURELESS技术。时隔3个月再次出现同样部位肺静脉狭窄,且狭窄段进一步向肺静脉远心端延伸,再次采用同样方法矫治,术后72小时仍不能脱离ECMO,患儿家属放弃治疗。共检索中文文献5篇,外文文献14篇,保留较为详细病例资料文献13篇,涉及病例98例。结论:术后肺静脉狭窄是肺静脉异位引流的严重并发症,属于进展性疾病,其发病可涉及多种机制,再手术病死率高,亦会发生再狭窄。SATURELESS技术在治疗肺静脉异位引流及术后肺静脉狭窄方面优点显著,但肺静脉切口如果反复出现因纤维内膜增生和瘢痕形成所导致的广泛肺静脉狭窄,则肺移植术或心肺移植成为此类患儿的唯一选择。Objective： To study clinical features and prognosis of pulmonary venous obstruction after repair of total anomalous pulmonary venous connection. Methods： A case of postoperative pulmonary vein stenosis after repair of scimitar syndrome was retrospectively analized and the related literature was reviewed.Results： Pulmonary vein stenosis occurred 6 months after operation of Scimitar syndrome. The thickening of the distal membrane of the pulmonary vein incision accompanied by stenosis was found during the first operation;the opening of pulmonary vein was enlarged by resection of thickened intima through satureless technique. 3 months later,pulmonary vein stenosis occurred once again at the same part. Family members gave up treatment at72 hours after reoperation,because the child couldn＇t be separated from ECMO. Related literature shows that satureless technique has significant advantages in the treatment of pulmonary venous ectopic drainage and postoperative pulmonary vein stenosis. Conclusion： postoperative pulmonary vein stenosis is a serious complication of pulmonary venous drainage and a progressive disease. It involve a variety of mechanisms and has high odds of reoperation and mortality. If pulmonary vein stenosis caused by fiber hyperplasia and scarring occurred many times at the incision of pulmonary vein incision,heart lung transplantation would become the only option for children.

关 键 词：弯刀综合征 肺静脉异位引流 肺静脉狭窄 

分 类 号：R54[医药卫生—心血管疾病]