Division of Congenital Heart Surgery, Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
World J Pediatr Congenit Heart Surg. 2024 Jul;15(4):472-480. doi: 10.1177/21501351241235959. Epub 2024 Apr 22.
Aortopulmonary window (APW) is a rare anomaly with variable morphology and associated cardiac anomalies. We evaluated impact of patient and operative factors on mid-term outcomes following APW repair. Twenty-nine patients underwent surgical APW repair at our institution from 1996 to 2022. Eight (28%) had simple APW, accompanied by only atrial septal defect or patent ductus arteriosus; 21 (72%) had complex APW with additional cardiovascular lesions, including nine with interrupted aortic arch. Median operative age was 19 days (range 2 days-1.5 years) via single-patch (n = 12, 41%), double-patch (n = 15, 52%), or ligation and division (n = 2, 7%). The only mortality occurred in-hospital 1.4 years postoperatively following remote myocardial infarction. Factors associated with longer postoperative length of stay were complex APW ( = .003), genetic syndrome ( = .003), noncardiovascular comorbidities ( = .002), lower birth weight ( = .03), and lower operative weight ( = .03). Six patients (21%) with complex APW underwent unplanned cardiothoracic reintervention(s), including two with arch reintervention following arch advancement for interruption. Reintervention-free survival was similar for simple versus complex APW, operative age categories, and repair techniques. At median follow-up 5.5 years postoperatively, no patients had residual APW or persistent pulmonary hypertension, 1 (3%) had greater than mild ventricular dysfunction, and 25 (89% survivors) had NYHA class I functional status. Operative APW repair has excellent mid-term survival, durability, and functional status, regardless of operative age, cardiovascular comorbidities, or repair technique. Cardiac and noncardiac comorbidities may be associated with prolonged length of stay.
主肺动脉窗(APW)是一种罕见的异常,具有不同的形态和相关的心脏异常。我们评估了患者和手术因素对 APW 修复后中期结果的影响。1996 年至 2022 年,我院 29 例患者接受了外科 APW 修复。8 例(28%)为单纯性 APW,仅伴有房间隔缺损或动脉导管未闭;21 例(72%)为复杂性 APW,伴有其他心血管病变,包括 9 例主动脉弓中断。中位手术年龄为 19 天(范围 2 天-1.5 年),采用单补丁(n=12,41%)、双补丁(n=15,52%)或结扎和分割(n=2,7%)。唯一的死亡发生在术后 1.4 年,为远程心肌梗死。与术后住院时间延长相关的因素包括复杂的 APW( = .003)、遗传综合征( = .003)、非心血管合并症( = .002)、出生体重较低( = .03)和手术体重较低( = .03)。6 例(21%)复杂性 APW 患者行计划性心胸再干预,其中 2 例因弓中断行弓部推进后行弓部再干预。单纯性 APW 与复杂性 APW、手术年龄组和修复技术的无再干预生存率相似。术后中位随访 5.5 年,无患者残留 APW 或持续性肺动脉高压,1 例(3%)有大于轻度心室功能障碍,25 例(89%幸存者)有 NYHA Ⅰ级功能状态。无论手术年龄、心血管合并症或修复技术如何,手术 APW 修复均具有极好的中期生存率、耐久性和功能状态。心脏和非心脏合并症可能与住院时间延长有关。