Wang Chenhan, Chen Bingjie, Chen Jingnan, Luo Jinwen, Yang Guangxian, Yi Liwen, Deng Xicheng
Department of Cardiothoracic Surgery, The Affiliated Children's Hospital of Xiangya School of Medicine, Central South University (Hunan Children's Hospital), Changsha, Hunan, China.
Front Pediatr. 2025 Aug 14;13:1622525. doi: 10.3389/fped.2025.1622525. eCollection 2025.
Double aortic arch (DAA) is a rare congenital vascular anomaly resulting in a complete vascular ring that encircles and compresses the trachea and esophagus, leading to respiratory and gastrointestinal symptoms. Accurate diagnosis and timely surgical intervention are essential for symptom relief and preventing complications. However, data on surgical outcomes and long-term follow-up are limited. This study retrospectively analyzed surgical outcomes and perioperative management of DAA to elucidate optimal diagnostic and therapeutic approaches.
A retrospective cohort study enrolled patients undergoing DAA repair (August 2015-May 2024). Participants were stratified into isolated DAA or DAA with associated intracardiac anomalies groups. Demographic, operative, and outcome variables were compared.
Among 10 patients undergoing double aortic arch repair, 6 comprised the isolated DAA group (3 males/3 females; mean age 3.70 ± 3.18 months; mean weight 6.28 ± 2.77 kg) and 4 had associated intracardiac anomalies (3 males/1 female; mean age 6.70 ± 6.12 months; mean weight 6.15 ± 3.59 kg). Isolated DAA patients and those with intracardiac anomalies showed no statistically significant differences in: symptom onset (28.17 ± 37.66d vs. 30.50 ± 41.96d), anatomic subtypes (dominant right arch 83% vs. 50%), extracardiac anomaly rates (50% vs. 75%), or clinical manifestations-respiratory (67% vs. 100%) and other systemic (17% vs. 75%) (all > 0.05). All procedures were successfully completed with significantly shorter operative time in the isolated group (104.83 ± 22.23 vs. 233.25 ± 38.55 min, < 0.001). No significant intergroup differences ( > 0.05) were observed in preoperative ventilation, blood loss, ventilator duration, Cardiac Intensive Care Unit stay, drainage duration, hospitalization, or complication rates. During mean 12.7-month follow-up (1-36 months), near-complete symptom resolution occurred in 9/10 survivors, with one death from respiratory failure in a comorbid patient 10 days post-discharge.
Surgical repair of double aortic arch demonstrates acceptable safety and efficacy in both infants and children, with favorable short-to-midterm clinical outcomes regardless of concomitant intracardiac anomalies.
双主动脉弓（DAA）是一种罕见的先天性血管异常，可形成一个完整的血管环，环绕并压迫气管和食管，导致呼吸和胃肠道症状。准确诊断和及时手术干预对于缓解症状和预防并发症至关重要。然而，关于手术结果和长期随访的数据有限。本研究回顾性分析了双主动脉弓的手术结果和围手术期管理，以阐明最佳的诊断和治疗方法。
一项回顾性队列研究纳入了接受双主动脉弓修复术的患者（2015年8月至2024年5月）。参与者被分为孤立性双主动脉弓组或合并心内异常的双主动脉弓组。比较了人口统计学、手术和结果变量。
在10例接受双主动脉弓修复术的患者中，6例为孤立性双主动脉弓组（3例男性/3例女性；平均年龄3.70±3.18个月；平均体重6.28±2.77kg），4例合并心内异常（3例男性/1例女性；平均年龄6.70±6.12个月；平均体重6.15±3.59kg）。孤立性双主动脉弓患者和合并心内异常的患者在以下方面无统计学显著差异：症状发作时间（28.17±37.66天 vs. 30.50±41.96天）、解剖亚型（右弓优势型83% vs. 50%）、心外异常发生率（50% vs. 75%）或临床表现——呼吸系统（67% vs. 100%）和其他全身系统（17% vs. 75%）（均P>0.05）。所有手术均成功完成，孤立组的手术时间明显更短（104.83±22.23 vs. 233.25±38.55分钟，P<0.001）。术前通气、失血量、呼吸机使用时间、心脏重症监护病房停留时间、引流时间、住院时间或并发症发生率在组间无显著差异（P>0.05）。在平均12.7个月的随访期（1至36个月）内，10名幸存者中有9名症状几乎完全缓解，1名合并症患者在出院后10天因呼吸衰竭死亡。
双主动脉弓的手术修复在婴儿和儿童中显示出可接受的安全性和有效性，无论是否合并心内异常，均具有良好的短期至中期临床结果。
