主动脉缩窄或主动脉弓中断合并心内畸形一期修复

目的 探讨采用经胸骨正中切口一期修复主动脉缩窄(CoA)或主动脉弓中断(IAA)合并心内畸形的治疗效果.方法 2002年7月至2009年6月,经胸骨正中切口行降主动脉远端和主动脉弓下缘端侧吻合术一期修复CoA或IAA合并心内畸形病儿43例,其中CoA 34例,IAA 9例(A型6例、B型3例),合并心内畸形包括室间隔缺损42例、动脉导管未闭34例、房间隔缺损12例、主动脉瓣下隔膜狭窄5例、二尖瓣关闭不全2例,右心室双出口1例.结果 手术死亡1例,为术后肺动脉高压和严重低心排血量综合征者.术后并发症包括严重低心排血量综合征3例,低氧血症6例,肺部炎症11例,肺不张14例,声音嘶哑19例,室上性心动过速23例.8例失访.34例随访3个月～5年,生活质量明显改善,心脏超声心动图和CT检查显示吻合口无明显再缩窄发生.结论 经胸骨正中切口,采用主动脉远端和主动脉弓下缘端侧吻合技术一期修复CoA或IAA合并心内畸形的手术早、中期效果良好,能明显减少术后再狭窄.

One-stage repair of aortic coarctation or interrupted aortic arch associated with cardiac anomalies through median sternotomy

Objective Study the management and outcomes of one-stage repair of aortic coarctation or interrupted aortic arch associated with cardiac anomalies through median sternotomy.Methods From July 2002 to June 2009,43 patients with aortic coarctation(34 cases)or interrupted aortic arch(9 cases)and associated with cardiac anomalies underwent one-stage repair.There were 27 males and 16 females.The age ranged from 5 months to 9 years and the body weight from 3.5 kg to 29.0 kg.The associated cardiac anomalies included ventricular septal defect in 42 patients,patent ductus arterious in 34,secundum atrial septal defect in 12,subaortic stenesis in 5,mitral valve regurgitation in 2 and double outlet of right vantricule in 1.All patients underwent one-stage repair through median sternotomy.The aortic continuity was reestablished by direct anastomosis between the descending aortic segment and aortic arch.Results There was one postoperative death.The causs was pulmonary hypertension and severe low cardiac output syndrome.The postoperative complications included severe low cardiac output syndrome in 3 patients,hypoxemia in 6,pneumonia in 11,atelectasis in 14,injury of recurrent laryngeal nerve in 19,and supra ventricular tachycardia in 23.34 patients were followed up from 3 months to 5 years and were in good condition without recoarctation.Conclusion The outcomes of early and medium term for one-stage repair of aortic coarctation or interrupted aortic arch and associated cardiac anomalies through median sternotomy is excellent.Technique of extended anastomosis between the descending aortic segment and aortic arch may reduce the incidence of recoarctation