摘要目的 总结经胸骨正中切口一期矫治主动脉缩窄或弓中断合并心内畸形的经验.方法 2007年1月-2008年7月手术治疗24例.包括主动脉缩窄9例,主动脉缩窄合并主动脉弓发育不良12例,主动脉弓中断3例.4例合并右室双出口(Traussig-Bing型).22例合并非限制性室间隔缺损,2例不合并室间隔缺损病儿1例合并主动脉瓣下狭窄,另l例合并肺静脉狭窄.主动脉弓降部成形均在深低温低流量持续性选择性脑灌注下进行.3例主动脉弓中断及9例主动脉缩窄病儿采用端端吻合术.12例主动脉缩窄合并主动脉弓发育不良病儿中采用扩大端端吻合术8例,端侧吻合术2例,补片成形术2例.结果 死亡2例.全组病儿围术期未出现神经系统并发症及肾功能损害.术后反复呼吸道感染2例.除l例残存压差大于20mm Hg外,最长随访18个月,尚未发现再缩窄发生.结论 主动脉缩窄或弓中断合并心内畸形一经诊断即需尽早手术.经胸骨正中切口一期矫治是安全、有效的.充分切除动脉导管组织,广泛彻底游离松解胸部各血管进行无张力吻合以及选择恰当的组织一组织吻合术式是主动脉弓降部成形手术成功及减少再缩窄发生的关键.

abstractsObjedtve To summarize the experience of single-stage repair of coarctation of aorta(CoA) or interrupted aortic arch (IAA)and associated intracardiac defects through median stemotomy.Methods From Jan 2007 to Jul 2008,a total of 24 pa-tients with CoA or IAA and associated intracardisc defects were surgically repaired in single-stage through median stermotomy,inchud-ing 9 coanctation of aorta,12 coarctation with aortic arch hypoplasia,and 3 interrupted aorlic arch,.The associated intracardiac de-fects were Taussing-Bing anomaly 4,non-restricted VSD 22,subaortic stenosis 1 and pulmonary vein stenosis 1.The age ranged form 1 to 99 months (average 16 months) and the body weight ranged from 4 to 19 kg(average 9.3 kg).Aortic arch reconstruction was performed by hypothermic continuous low flow bypass using regional perfusion for all patients.Three patients with LAA and 9 patients with CoA underwent end-to-end ansetomosis.Of the 12 patients with coarctation and aortic arch hyipoplasia,8 patiellts underwent ex-tended end-to-end anastomosis,2 patients underwent end-to-side anastomosis and 2 patients underwent aortoplasfy.Results 2 cases were dead. One infant with Taussig-Bing type heart was dead of severe infection after 47 days postoperative,the other one who associ-ated with LAA and VSD dead of pulmonary hypertension crisis due to pneumonia after 15 days postoperative.No patient presented neu-rdogieal complication and renal insufficiency during the perioperation.2 cases presented recurrent respiratory problem.During the 18months follow-up,no patient presented with recoarctation except one with pressure gradient more than 20 mm Hg.Conclusion Pa-tients with coarctation of aorta or interrupted aortic arch and associsted intracardisc defects should be surgically treated as early as pos-sible when diagnosis was mode.Single-stage sortic arch reconstruction through median stemotomy using continuous regional perfusion is an effective and safe procedurd.Sufficient resection of ductus,extensive dissection of thoracic vessels and optimal tissus-tissue anas-tomosis techmique are very important for successful repair and avoiding recoarctation.