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目的回顾总结先天性主动脉弓中断及合并畸形一期手术治疗经验。方法1988年7月至2004年8月,手术治疗主动脉弓中断及合并畸形20例。男12例,女8例;手术年龄18d～9岁。其中<1岁者10例,平均年龄(3.37±2.45)个月,平均体重(5.33±1.72)kg;>1岁者10例,平均年龄(2.60±1.24)岁,平均体重(13.10±6.53)kg。所有病儿术前均有反复呼吸道感染史。A型15例,B型5例。2例经双切口手术,即先通过左后外侧切口纠治主动脉弓中断,再经正中切口纠治合并畸形。18例采用正中切口同时纠治主动脉弓中断和合并畸形。结果全组死亡3例,死于术后严重肺动脉高压危象2例、严重心功能衰竭1例。17例随访3个月～12年,均恢复良好。结论主动脉弓中断采用正中切口方法,操作简便,暴露清楚,创伤小,有利于术后恢复。本病早期即出现肺动脉高压,临床上一经确诊宜尽早手术。
Objective To summarize the experience of one-stage surgical treatment of congenital aortic arch disruption and combined deformity. Methods From July 1988 to August 2004, 20 cases of aortic arch interrupted and combined deformity were treated surgically. There were 12 males and 8 females. The operative age ranged from 18 days to 9 years. The average age was (3.37 ± 2.45) months and the average body weight was 5.33 ± 1.72 kg. The average age was (2.60 ± 1.24) years old and average body weight was (13.10 ± 6.53) kg. All sick children had recurrent respiratory tract infection before surgery. A type in 15 cases, B type in 5 cases. 2 cases of double incision surgery, that is, first through the left posterolateral incision to correct the aortic arch interrupted by the median incision and correction of deformity. Eighteen patients underwent median incision to correct the aortic arch interruption and combined deformity. Results The whole group died of 3 cases, died of postoperative severe pulmonary hypertension crisis in 2 cases, severe heart failure in 1 case. 17 cases were followed up for 3 months to 12 years, all recovered well. Conclusion The aortic arch interrupted by the median incision method, simple operation, clear exposure, less trauma, is conducive to postoperative recovery. Pulmonary hypertension appears early in this disease, clinically diagnosed as soon as possible after surgery.