婴幼儿主动脉缩窄合并心内复杂畸形的一期手术治疗

One-Stage Repair of Coarctation of Aorta in Neonates and Infants Associated With Complex Cardiac Anomalies

目的:探讨婴幼儿主动脉缩窄合并心内复杂畸形一期手术的治疗方法及手术效果。方法:回顾分析2002-11至2008-09收治的婴幼儿主动脉缩窄合并心内复杂畸形一期手术治疗的临床病例。共计18例,其中男12例,女6例。年龄平均(7.7±8.8)个月,体重(6.1±2.4)kg。主动脉缩窄包括合并主动脉弓发育不良5例,单纯主动脉缩窄13例。不合并主动脉弓发育不良者,切除缩窄段,端端吻合降主动脉;合并主动脉弓发育不良者,切除缩窄段,用自体心包或自体肺动脉片加宽主动脉弓。心内复杂畸形的矫治包括完全性大动脉转位大动脉调转术8例,右心室双出口根治术6例,右心室双出口陶西平畸形大动脉调转术3例,完全性心内膜垫缺损矫治术1例。结果:所有患儿出院前超声心动图检查均显示主动脉弓降部缩窄解除,吻合口通畅,血流正常。无中枢神经系统并发症。术后早期上、下肢收缩压基本相等者4例,上肢高于下肢15～20mmHg者8例,上肢低于下肢收缩压5～10mmHg者6例。死亡3例,死亡率16.7%。1例术后当天死于低心排综合征,1例术后9天死于肺部感染,1例术后7天死于突发恶性心律失常。术后另3例出现右侧膈肌麻痹行膈肌折叠术。结论:婴幼儿主动脉缩窄合并心内复杂畸形经胸骨正中切口一期手术治疗,主动脉弓及降主动脉上段显露良好。同期矫治心内复杂畸形手术效果满意。深低温停循环区域性脑灌注可有效降低神经系统并发症的发生。

Objective ：To summarize the clinical experience of one-stage repair of coarctation of the aorta in neonates and infants associated with complex cardiac anomalies. Methods：There were 18 patients with coarctation of the aorta associated with complex cardiac anomalies from November 2002 to September 2008 were surgically repaired. Patients age from 20 days to 3 years（7.7 ±8. 8 months） ,with the mean body weight from 3.8 kg to 13 kg（6. 1 ± 2. 4 kg）. All patients were one-stage repaired through median sternotomy. Coarctation of the aorta consisted of hypoplastic aortic arch in 5 cases and single coarctation of the aorta in 13. After widely mobilization and disassociation of brachial arteries, aortic arch and upper part of descending aorta, end to end anastomosis of aorta with removal of the coarctated segment was performed for patients with coarctation of the aorta, and aortic arch was widened with autologous pericardial patch for patients with hypoplasia of aortic arch. The concomitant procedures consisted of arterial switch operation in 11 patients who were associated with transposition of great artery or Tausing-Bing anomaly. Results：There were 3 in-hospital deaths for low cardiac output syndrome, pulmonary infection and fatal arrhythmia respectively. No complication of the central nervous system was found. There were no obvious systolic pressure difference between arm and leg in 4 patients. The systolic pressure difference between lower limb and upper limb measured after operation was 2 kpa to 3 kpa in 8 patients. The systolic pressure of upper limb was higher than that of lower limb in 6 patients and the pressure difference was 0. 67 kpa to 1.33 kpa. The echocardiography revealed no residual aortic arch obstruction. Conclusion：The aortic arch and upper part of the descending aorta could be well exposed through median sternotomy. The short-term result of one-stage repair of coarctation of the aorta in neonates and infants associated with complex cardiac anomalies was excellent. Deep hypothermic circulatory arrest combined with regional cerebral perfusion could protect the central nervous system during the operation.