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大动脉调转术治疗法洛四联症合并主动脉弓缩窄及主动脉弓发育不良

Arterial Switch Operation in Patients With Taussig-Bing Anomaly and Aortic Arch Obstruction.

机构信息

Department of Cardiothoracic Surgery, The Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.

Department of Cardiothoracic Surgery, The Royal Children's Hospital, Melbourne, Australia.

出版信息

Ann Thorac Surg. 2022 Sep;114(3):834-840. doi: 10.1016/j.athoracsur.2021.05.082. Epub 2021 Jun 29.

Abstract

BACKGROUND

Arterial switch operation (ASO) in children with Taussig-Bing anomaly and associated aortic arch obstruction (AAO) is technically demanding. This study sought to determine outcomes of patients with Taussig-Bing anomaly and AAO who underwent ASO at a single institution.

METHODS

From 1983 to 2015, 844 patients underwent an ASO for biventricular repair at The Royal Children's Hospital. Twenty-eight patients (3.3%) had Taussig-Bing anomaly and AAO.

RESULTS

Of 28 patients, 21 (75%) underwent ASO and AAO repair as a single-stage procedure and 7 (25%) had arch repair before ASO. There were no early or late deaths. Follow-up after hospital discharge was available for all patients. Median follow-up was 17.5 years (interquartile range, 9.2-22.3; range, 4.0-31.5). All patients were in New York Heart Association class I at last follow-up. Freedom from reintervention was 61% (95% confidence interval, 40-76) and 56% (95% confidence interval, 36-73) at 5 and 15 years, respectively, after ASO. The most common reinterventions were repair of recurrent AAO, branch pulmonary artery stenosis, and neoaortic valve repair. Four of 28 patients (14%) required 5 reinterventions (3 reoperations and 2 catheter reintervention) for recurrent AAO at a median of 2.2 years after ASO (interquartile range, 2.1-2.3).

CONCLUSIONS

Patients with Taussig-Bing anomaly and AAO can undergo ASO with excellent survival. Reintervention is equally common for both left- and right-sided obstruction.

摘要

背景

动脉调转术(ASO)治疗法洛四联症合并主动脉弓缩窄(AAO)技术要求较高。本研究旨在探讨单一中心接受 ASO 治疗的法洛四联症合并 AAO 患者的结局。

方法

1983 年至 2015 年,共有 844 例患者在皇家儿童医院接受了双心室修复术,其中 28 例(3.3%)患有法洛四联症合并 AAO。

结果

28 例患者中,21 例(75%)患者接受了 ASO 和 AAO 修复的单阶段手术,7 例(25%)患者先进行了弓部修复。无早期或晚期死亡。所有患者均获得了出院后的随访。中位随访时间为 17.5 年(四分位距,9.2-22.3;范围,4.0-31.5)。末次随访时,所有患者纽约心功能分级均为 I 级。ASO 后 5 年和 15 年无再次干预的生存率分别为 61%(95%置信区间,40-76)和 56%(95%置信区间,36-73)。最常见的再次干预是 AAO 复发、肺动脉分支狭窄和新主动脉瓣修复。28 例患者中有 4 例(14%)在 ASO 后 2.2 年(四分位距,2.1-2.3)因 AAO 复发需要 5 次再次干预(3 次再次手术和 2 次介入治疗)。

结论

法洛四联症合并 AAO 患者可接受 ASO 治疗,且生存率较高。左、右心病变患者的再次干预发生率相当。

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