主动脉弓中断的手术治疗

Surgical repair of interrupted aortic arch

目的 回顾总结主动脉弓中断的手术治疗。方法 1998年 10月～ 2 0 0 0年 9月 ,手术治疗主动脉弓中断 6例。男性 5例 ,女性 1例 ,手术年龄 2～ 9岁 ,体重 10 .5～ 31.0kg。所有病例术前均有反复呼吸道感染史 ,病例分型A型 4例 ,B型 1例 ,另 1例为主动脉第 5弓残存伴狭窄。 2例经双切口方法手术 ,即通过左后外侧切口纠治主动脉弓中断 ,再经正中切口纠治心内畸形。 4例均采用正中切口方法纠治主动脉弓中断和心内畸形。结果 全组死亡 2例 ,均为术后发生严重肺动脉高压危象死亡。 4例随访 ,最长 12年 ,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 ,创面小 ,有利于术后恢复。由于该病早期出现肺动脉高压 ,一经诊断应及时手术纠治 ,手术在 1岁内进行为好。

Objective Summarize and review on surgical repair of interrupted aortic arch (IAA). Methods Between October 1998 and September 2000, six patients (male 5, female 1) with IAA were operated. The operative age was 1.8-9.0 years (mean 3.96±2.56) and body weight was 10.5-31.0 kg(mean 14.7±57.89). All patients had respiratory infection repeatedly, 4 were IAA type A, one was type B and one was the fifth aortic arch remnants with stenosis. The operations were performaed by two separated incisions in 2 patients, the IAA was repaired through lateral intercostal space incision and the ventricular septal defect was repaired through midline sternotomy. But now, the IAA and ventricular septal defect were all repaired through midline sternotomy. Results There were two deaths as a result of severe pulmomnary hypertension crisis. Four patients were followed up, the results was excellent. Conclusion IAA operation can achieve good exposure, less incisional wound and excellent recovery through midline sternotomy. Because of pulmonary hypertension the operation should be performed as early as possible, the best operative age was within 1 year of life.