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大动脉转位伴主动脉弓梗阻一期修复术后的结果和再干预。

Outcomes and re-interventions after one-stage repair of transposition of great arteries and aortic arch obstruction.

机构信息

Department of Cardiovascular Surgery, University Hospital Bern, Inselspital, Switzerland.

出版信息

Eur J Cardiothorac Surg. 2011 Feb;39(2):213-20. doi: 10.1016/j.ejcts.2010.05.009. Epub 2010 Jul 1.

Abstract

OBJECTIVES

One-stage repair of transposition of great arteries (TGA) and aortic arch obstruction (AAO) is currently advocated, but carries formidable surgical challenges. This report presents our experience and re-interventions for residual lesions over the last 10 years.

METHODS

Twenty-two patients (19.5 ± 42.4 days; range 2-206; median 10 days, 3.5 ± 0.6 kg) diagnosed with TGA (nine patients) or double outlet right ventricle (DORV) (13 patients) and AAO underwent one-stage repair. Of the nine TGA patients (two with intact ventricular septum), AAO were: two patients hypoplastic arch, one patient discrete coarctation, four patients hypoplastic arch with coarctation and two patients interrupted aortic arch. The 13 DORV patients were all of Taussig-Bing type and one showed multiple ventricular septal defects (VSDs). The degree of AAO ranged from hypoplastic arch in five patients, coarctation two patients, combined four patients and interrupted aortic arch (IAA) two patients. Arterial switch with Lecomte ± VSD repair was performed during cooling, and aortic arch repair was performed under deep hypothermic circulatory arrest (DHCA) (35 ± 14 min at 16.9 ± 0.7 °C). Our preference was to use homograft patch-plasty for arch and direct end-to-side anastomosis for coarctation repair. Aortic-cross-clamp time was 124 ± 24 min and cardiopulmonary bypass (CPB) time 215 ± 84 min.

RESULTS

Early survival was 19/22 (86%) up to 30 days without mortality in the second half of our series. Three patients required extracorporeal membrane oxygenation (ECMO) support and renal support was needed in three and preferred permanent pace maker (PPM) implantation in two. Length of stay was 21.9 ± 22.1 days. There was one late death and overall survival was 18/22 (82%) for the follow-up period of 4.8 years (0.2-9.8 years). Eight patients (44%) required re-intervention for re-coarctation. Four patients required right ventricular outflow tract (RVOT)/pulmonary artery re-interventions. At follow-up, there was no requirement for aortic valve replacement, residual VSD closure and no evidence of ventricular dysfunction.

CONCLUSIONS

One-stage repair of TGA/DORV and AAO can be performed safely with a good survival rate. Three important lessons that we have learnt are as follows: (1) the subpulmonary VSD may have a perimembraneous component, (2) late re-coarctation is not infrequent and (3) late residual right-sided cardiac lesions remain an issue in complex TGA repair.

摘要

目的

目前提倡对大动脉转位(TGA)和主动脉弓阻塞(AAO)进行一期修复,但这带来了巨大的手术挑战。本报告介绍了我们在过去 10 年中治疗残余病变的经验和再次介入治疗。

方法

22 例(出生后 19.5±42.4 天;年龄 2-206 天;中位年龄 10 天,体重 3.5±0.6kg)诊断为 TGA(9 例)或右心室双出口(DORV)(13 例)和 AAO 的患者接受了一期修复。在 9 例 TGA 患者(2 例室间隔完整)中,AAO 为:2 例患者主动脉弓发育不良,1 例患者主动脉弓狭窄,4 例患者主动脉弓发育不良伴狭窄,2 例患者主动脉弓中断。13 例 DORV 患者均为 Taussig-Bing 型,1 例患者存在多发性室间隔缺损(VSD)。AAO 的严重程度为 5 例患者主动脉弓发育不良,2 例患者主动脉弓狭窄,4 例患者主动脉弓发育不良伴狭窄,2 例患者主动脉弓中断。在降温过程中进行动脉转换和 Lecomte+VSD 修复,在深低温循环停止(DHCA)下进行主动脉弓修复(16.9±0.7°C 时 35±14 分钟)。我们倾向于使用同种异体移植物补片修补弓部,直接端侧吻合修复缩窄部。主动脉阻断时间为 124±24 分钟,体外循环(CPB)时间为 215±84 分钟。

结果

早期存活率为 22 例中的 19 例(86%),直到第 2 半段无死亡。3 例患者需要体外膜氧合(ECMO)支持,3 例患者需要肾脏支持,2 例患者需要永久性起搏器(PPM)植入。住院时间为 21.9±22.1 天。有 1 例患者死于晚期,在随访 4.8 年(0.2-9.8 年)期间的总存活率为 18/22(82%)。8 例(44%)患者需要再次介入治疗以纠正再狭窄。4 例患者需要右心室流出道(RVOT)/肺动脉再次介入治疗。随访时,主动脉瓣置换、残余 VSD 关闭和心室功能障碍均无需治疗。

结论

TGA/DORV 和 AAO 的一期修复是安全的,有较好的生存率。我们学到了三个重要的经验:(1)肺动脉瓣下室间隔缺损可能有膜周成分,(2)晚期再狭窄并不少见,(3)复杂 TGA 修复后右心残余病变仍然是一个问题。

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