Buckley Jason R, Costello John M, Smerling Arthur J, Sassalos Peter, Amula Venu, Cashen Katherine, Riley Christine M, Bakar Adnan M, Iliopoulos Ilias, Jennings Aimee, Narasimhulu Sukumar Suguna, Mastropietro Christopher W
Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina.
Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina.
Ann Thorac Surg. 2023 Jan;115(1):144-150. doi: 10.1016/j.athoracsur.2022.08.040. Epub 2022 Sep 6.
Truncus arteriosus with interrupted aortic arch (TA-IAA) is a rare congenital heart defect with historically poor outcomes. Contemporary multicenter data are limited.
A retrospective cohort study of children who underwent repair of TA-IAA between 2009 and 2016 at 12 tertiary care referral centers within the United States was performed. Major adverse cardiac events (MACE) were defined as postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. TA-IAA patients were compared with TA patients who underwent repair during the study period from the same institutions.
We reviewed 35 patients with TA-IAA. MACE occurred in 12 patients (34%). Improvement over time was observed during the study period with 11 events (92%) occurring in the first half of the study period (P = .03). Factors associated with MACE included moderate or severe truncal valve insufficiency (P < .01), concomitant truncal valve repair (P = .04), and longer cardiopulmonary bypass duration (P = .02). In comparison with 216 patients who underwent TA repair, patients with TA-IAA had a higher rate of MACE, but this finding was not statistically significant (34% vs 20%, respectively; P = .07). Additionally no differences between TA-IAA and TA groups were observed for unplanned reoperations (14% vs 22%, respectively; P = .3), hospital length of stay (24 vs 23 days, P = .65), or late deaths (7% vs 7%, P = 1.00).
In this contemporary, multicenter cohort the rate of MACE after repair of TA-IAA was high but improved during the study period. Early childhood outcomes of patients with TA-IAA were similar to those with TA.
永存动脉干合并主动脉弓中断（TA-IAA）是一种罕见的先天性心脏缺陷，历来预后较差。当代多中心数据有限。
对2009年至2016年在美国12家三级医疗转诊中心接受TA-IAA修复手术的儿童进行回顾性队列研究。主要不良心脏事件（MACE）定义为术后体外膜肺氧合、心肺复苏或手术死亡。将TA-IAA患者与同期在同一机构接受修复手术的TA患者进行比较。
我们回顾了35例TA-IAA患者。12例患者（34%）发生了MACE。在研究期间观察到随着时间推移有所改善，11例事件（92%）发生在研究期的前半段（P = 0.03）。与MACE相关的因素包括中度或重度动脉干瓣膜关闭不全（P < 0.01）、同期动脉干瓣膜修复（P = 0.04）和体外循环时间延长（P = 0.02）。与216例接受TA修复的患者相比，TA-IAA患者的MACE发生率更高，但这一发现无统计学意义（分别为34%和20%；P = 0.07）。此外，TA-IAA组和TA组在计划外再次手术（分别为14%和22%；P = 0.3）、住院时间（24天对23天，P = 0.65）或晚期死亡（7%对7%，P = 1.00）方面未观察到差异。
在这个当代多中心队列中，TA-IAA修复术后的MACE发生率较高，但在研究期间有所改善。TA-IAA患者的儿童早期结局与TA患者相似。
