Department of Pediatric Cardiology, Mackay Memorial Hospital, Taipei City, Taiwan.
Department of Critical Care, Mackay Memorial Hospital, Taipei City, Taiwan.
Am J Case Rep. 2023 Dec 21;24:e942193. doi: 10.12659/AJCR.942193.
BACKGROUND An aortopulmonary window (APW) is an uncommon congenital defect of the septation between the ascending aorta and pulmonary trunk. The combination of APW and interrupted aortic arch (IAA) is even rarer, with the hallmark characteristics of high peri-operative mortality and postoperative obstruction of the aortic arch, pulmonary artery, and left main bronchus. These complications often need re-interventions. CASE REPORT We present 2 cases with diagnoses of APW and IAA that were treated with single-stage repair. Case 1: A male 32-week premature newborn (weight 1789 g) had APW type I and IAA type A. He had severe postoperative aortic arch obstruction on postoperative day 1, and we re-intervened promptly. He was still asymptomatic after 6 years. Case 2: A male term neonate had APW type III and IAA type A. He had left vocal cord paralysis and left bronchial compression postoperatively. We applied prolonged noninvasive respiratory supports. The complications resolved without re-intervention on postoperative day 66. Progressive arch stenosis at anastomosis after operation required close follow-up with echocardiography. CONCLUSIONS These 2 reports highlight the feasibility of single-stage surgical repair while addressing 2 challenges: (1) Recurrent arch stenosis: Lower body weight and direct end-to-side anastomosis without patch augmentation could be risk factors for re-intervention. (2) Bronchial compression: Presentation of the second reported case implied that bronchial compression may not warrant immediate re-intervention unless there is complete obstruction, persistent atelectasis, or recurrent infection. Further studies on long-term outcomes of different surgical procedure would help us to clarify the proper way to avoid re-intervention.
主动脉肺动脉窗(APW)是升主动脉和肺动脉干之间分隔不全的一种罕见先天性缺陷。APW 与主动脉弓中断(IAA)的联合更为罕见,其特征性表现为围手术期死亡率高,术后主动脉弓、肺动脉和左主支气管阻塞。这些并发症常需要再次介入治疗。
我们报告了 2 例诊断为 APW 和 IAA 的病例,均采用一期修复治疗。病例 1:一名 32 周早产男性新生儿(体重 1789 克)患有 I 型 APW 和 A 型 IAA。术后第 1 天出现严重的主动脉弓阻塞,我们及时进行了再次介入治疗。6 年后他仍无症状。病例 2:一名足月男性新生儿患有 III 型 APW 和 A 型 IAA。术后出现左侧声带麻痹和左侧支气管受压。我们应用了长时间的无创呼吸支持。术后第 66 天,并发症无需再次介入治疗而自行缓解。术后吻合口处弓部狭窄进展需要密切随访心脏超声。
这 2 例报告强调了一期手术修复的可行性,同时解决了 2 个挑战:(1)再狭窄:体重较低和直接端侧吻合而不使用补片增强可能是再次介入治疗的危险因素。(2)支气管压迫:第二例报告的病例提示,除非出现完全阻塞、持续肺不张或反复感染,否则支气管压迫不一定需要立即再次介入治疗。进一步研究不同手术方式的长期结局有助于阐明避免再次介入治疗的恰当方法。