临床表现明显的血管环伴先天性气管狭窄患儿的长期预后

作     者：Chiu P.P.L. Rusan M. Williams W.G. P.C.W. Kim 刘凯 

作者机构：Division of General SurgeryHospital for Sick ChildrenUniversity of TorontoTorontoOnt. M5G 1X8Canada 

出 版 物：《世界核心医学期刊文摘（儿科学分册）》 (Dkgest of the World Latest Medical Information)

年 卷 期：2006年第2卷第9期

页      面：20-20页

学科分类：1002[医学-临床医学] 100202[医学-儿科学] 10[医学] 

主　　题：气管狭窄 血管环 心脏畸形 修复术 总体死亡率 联合修复 继发性疾病 个体特点 修复手术 

摘      要：Aim: Congenital tracheal stenosis (CTS) associated with vascular rings (VRs) is considered a secondary condition,but relative indications for selective VRs or combined VR and CTS repairs are unclear. Here,we report long-term outcomes after VR repair associated with tracheal narrowing (TN). Method: Retrospective analysis of patients admitted to a single institution with VR from 1982 to 2004. Of 131 patients with VR (Cardiac Registry Database),29 (19 males,10 females; age range,0 days-4 years) associated with symptomatic TN. Patients were followed until age 18 years. Results: One hundred thirty-one patients with VR were categorized into 3 treatment groups. There were 102 patients with VR with no symptomatic TN treated by VR repair (4 deaths,4% mortality). Of the 29 patients with VR + TN symptoms,16 were treated by selective VR repair (mean age,5.3 months; range,17-288 days) with 3 deaths (19% mortality) and 13 underwent combined VR and CTS repairs (mean age,11 months; range,1 day-4 years) with 6 deaths (46% mortality),for an overall mortality of 31% (P 0.01,χ 2 test). Of the 102 patients with VR,17 had intracardiac anomalies with 3 deaths (18% mortality). In contrast,7 of 29 patients with VR with TN and intracardiac anomalies died 4(57% mortality); 3 patients in combined cardiac and CTS repairs (2 deaths,67% mortality) and 4 in selective VR repairs (2 deaths,50% mortality). Conclusions: Not all cases of TN associated with VR require combined repairs. The presence of TN and/or intracardiac anomalies in patients with VR,however,increases mortality risk to the repair of VR. Surgical decision requires individualized clinical pathway.