Deraz Salem Elsayed, Elnady Hamza
Department of Pediatrics, Faculty of Medicine, Menoufia University, Shebeen El-Kom, Egypt.
Department of Pediatrics, Fujairah Hospital, Emirates Health Services, Fujairah, United Arab Emirates.
Ann Pediatr Cardiol. 2025 Mar-Apr;18(2):93-99. doi: 10.4103/apc.apc_27_25. Epub 2025 Aug 29.
Conventionally, the surgical approach for Taussig-Bing anomaly (TBA) and transposition of the great arteries with aortic arch obstruction (AAO) has often involved a two-stage strategy. However, in contemporary practice, most centers now favor a one-stage repair. Despite this trend, the choice between one-stage and two-stage strategies may still arise in specific anatomical cases, such as those with interrupted AA (IAA) or in low-weight infants.
We conducted a review of our institutional experience with single-stage correction in 24 consecutive cases of TBA associated with AAO. Each patient underwent a single-stage correction that included an arterial switch operation (ASO) along with ventricular septal defect (VSD) closure through baffling of the left ventricle to the neo-aorta and AA repair. Early mortality or reoperation was defined as death or the need for reoperation occurring prior to hospital discharge or within 30 days following ASO. Reintervention was classified as either a reoperation or a transcatheter procedure.
The study comprised 24 cases. Among them, 6 (25%) patients had TBA with aortic coarctation, 15 (62.5%) patients had TBA with hypoplastic AA (HAA), 2 (8.3%) patients presented with TBA and IAA, and one patient had TBA with HAA alongside partial anomalous pulmonary venous connection. There were two early postoperative deaths (8.3%). Recoarctation occurred in 2 (8.3%) cases, both of which involved TBA with AA hypoplasia. Both patients successfully underwent balloon dilation of the coarctation after 285 and 312 days, respectively. One (4.2%) patient developed subaortic stenosis and underwent a modified Konno procedure after 233 days. The total number of reinterventions was 5 (20.8%), which included three cardiac catheterization procedures (two successful balloon dilations of the coarctation and one unsuccessful balloon dilation of the supravalvular stenosis), along with two surgical reinterventions.
The single-stage repair involving VSD closure, AA reconstruction, and ASO is an applicable and safe option for patients with combined TBA and concomitant AAO. We recorded reinterventions for recurrent coarctation, supravalvular, and subvalvular aortic stenosis. Long-term follow-up is essential, and early percutaneous interventions may help lower the rates of repeat surgeries.
传统上，治疗陶西格-宾畸形（TBA）和合并主动脉弓梗阻（AAO）的大动脉转位时，手术方法通常采用两阶段策略。然而，在当代实践中，大多数中心现在倾向于一期修复。尽管有这种趋势，但在特定的解剖病例中，例如那些伴有主动脉弓中断（IAA）的病例或低体重婴儿，仍可能需要在一期和两阶段策略之间做出选择。
我们回顾了本院连续24例TBA合并AAO患者的一期矫正经验。每位患者均接受了一期矫正，包括动脉调转术（ASO），以及通过将左心室与新主动脉进行分流来闭合室间隔缺损（VSD）和修复AA。早期死亡率或再次手术定义为在出院前或ASO后30天内发生的死亡或再次手术的需要。再次干预分为再次手术或经导管介入治疗。
该研究包括24例患者。其中，6例（25%）患者为TBA合并主动脉缩窄，15例（62.5%）患者为TBA合并主动脉弓发育不全（HAA），2例（8.3%）患者为TBA合并IAA，1例患者为TBA合并HAA及部分肺静脉异位连接。术后早期死亡2例（8.3%）。2例（8.3%）发生再缩窄，均为TBA合并主动脉弓发育不全。这两名患者分别在285天和312天后成功接受了缩窄部位的球囊扩张。1例（4.2%）患者发生主动脉瓣下狭窄，并在233天后接受了改良Konno手术。再次干预总数为5例（20.8%），包括3例心导管检查（2例缩窄部位球囊扩张成功，1例瓣上狭窄球囊扩张失败），以及2例外科再次干预。
对于合并TBA和AAO的患者，涉及VSD闭合、AA重建和ASO的一期修复是一种可行且安全的选择。我们记录了因复发性缩窄、瓣上和瓣下主动脉狭窄而进行的再次干预。长期随访至关重要，早期经皮介入治疗可能有助于降低再次手术率。
