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分期手术修复主动脉弓中断的婴儿。

Staged surgical repair for infants with interrupted aortic arch.

机构信息

Division of Cardiovascular Surgery, Department of Surgery, MacKay Memorial Hospital, Taiwan.

Division of Cardiovascular Surgery, Department of Surgery, MacKay Memorial Hospital, Taiwan.

出版信息

Asian J Surg. 2020 Nov;43(11):1074-1077. doi: 10.1016/j.asjsur.2020.02.013. Epub 2020 Mar 14.

Abstract

BACKGROUND

Interrupted aortic arch (IAA) is a rare congenital cardiac anomaly, which necessitates surgical treatment. There are several surgical strategies for corrective repair of IAA, such as one-stage repair, rapid two-stage repair and two-stage repair. Here, we reported our surgical result of staged-repair policy for the patients with IAA.

METHOD

From November 2003 to July 2015, there were 14 patients (8 boys, 6 girls) with IAA treated by us. Except one teenager patient, we routinely used intravenous infusion of prostaglandin E1 for all the infant patients (n = 13) to keep adequate end organ perfusion before the first surgical intervention. Surgical repair was performed after the perfusion of end organs recovered.

RESULT

Two patients (1 teenager and 1 infant with one-stage surgery) were excluded from this study. At the time of the first surgery, we did the first-stage surgery with anastomosis in between aortic arch and descending aorta, division of patent ductus arteriosus and banding of pulmonary trunk through left thoracotomy. The overall surgical survival rate of the first surgery was 100% (12/12). At the time of the second surgery, corrective repair was done under cardiopulmonary bypass through median sternotomy. The surgical survival rate of the corrective surgery was also 100%. There is no late death during follow-up for 9 years (range 4.2-15.0 years).

CONCLUSION

Out of several surgical strategies for the infants with IAA, staged repair still could be a treatment option to achieve satisfied surgical result.

摘要

背景

主动脉缩窄(IAA)是一种罕见的先天性心脏畸形,需要手术治疗。IAA 的矫正修复有几种手术策略,如一期修复、快速两期修复和两期修复。在这里,我们报告了我们对 IAA 患者进行分期修复策略的手术结果。

方法

2003 年 11 月至 2015 年 7 月,我们治疗了 14 例 IAA 患者(8 名男孩,6 名女孩)。除了一名青少年患者外,我们常规对所有婴儿患者(n=13)静脉输注前列腺素 E1,以在第一次手术干预前保持终末器官充分灌注。在终末器官灌注恢复后进行手术修复。

结果

有 2 名患者(1 名青少年和 1 名婴儿接受一期手术)被排除在本研究之外。在第一次手术时,我们通过左开胸术在主动脉弓和降主动脉之间进行吻合、动脉导管切开术和肺动脉干带环术,进行一期手术。第一次手术的总体手术存活率为 100%(12/12)。在第二次手术时,通过正中开胸术在体外循环下进行矫正修复。矫正手术的手术存活率也为 100%。在 9 年(4.2-15.0 年)的随访中没有晚期死亡。

结论

在 IAA 婴儿的几种手术策略中,分期修复仍然是一种可以达到满意手术效果的治疗选择。

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