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

来源 :世界核心医学期刊文摘(儿科学分册) | 被引量 : 0次 | 上传用户:lili1006
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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. 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 There were 102 patients with VR with no sympathy with TN 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 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, Increasing mortality risk to the repair of VR. Surgical decision requires individualized clinical pathway.
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