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Abstract：
Introduction A double aortic arch (DAA) is a rare congenital vascular anomaly that encircles the trachea and esophagus, resulting in compression of both structures and causing variable symptoms of wheezing, stridor, increased work of breathing, or dysphagia. DAA usually presents in infancy but can be incidentally found later in life. The standard management of DAA is surgical repair. However, observation and follow-up have been recommended in asymptomatic or mild cases. The long-term outcome of surgical repair versus observation is not well-reported. We described the long-term clinical outcome of patients with DAA who were surgically repaired versus non-repaired at our institution. Methods Electronic medical records were searched for the patients diagnosed with DAA before the age of 18 years. Data from clinical, radiological, and bronchoscopic findings, pulmonary function test (PFT), and cardiopulmonary exercise testing (CPET) were extracted. A structured phone questionnaire of patients\' parents regarding past and current symptoms was also conducted. Results A total of 12 patients (eight males four females) with DAA were identified. Median age was 8.5 (1.5-17) years. The age at diagnosis was 60 (1-192) months. Post diagnosis follow-up period was 20 (2-156) months. Five patients were surgically repaired, and seven patients were not repaired. The median age of surgery was five (1-15) years in repaired patients. The phone questionnaire was completed in only 10 patients (five repaired and five non-repaired). Respiratory symptoms in infancy were reported in all repaired and non-repaired patients and were resolved in all five repaired patients and in four of the five non-repaired patients. One non-repaired patient complained of intermittent dyspnea on exertion. Gastrointestinal symptoms were present in infancy in three repaired and three non-repaired patients and were improved in two repaired and one non-repaired patient. PFT was performed in five patients (one repaired, four non-repaired) and showed normal forced expiratory volume in one second (FEV1), forced vital capacity (FVC), and FEV1/FVC in all patients. Low peak expiratory flow (PEF) was seen in the repaired patient and in three of the non-repaired patients. CPET was conducted in four non-repaired patients and showed maximal oxygen consumption (VO2-max) of 66% predicted (58-88), maximal ventilation (VE-max) of 75% predicted (70-104), and ventilatory reserve of 55% predicted (48-104). Conclusion Long-term clinical outcome is favorable in both repaired and non-repaired patients with DAA even though both groups reported respiratory symptoms during infancy. Therefore, clinical observation is a legitimate option in certain DAA patients.
摘要：
双主动脉弓（DAA）是一种罕见的先天性血管异常，围绕气管和食道，导致两个结构受压，并引起喘息的各种症状，stridor,增加呼吸工作，或者吞咽困难.DAA通常在婴儿期出现，但可以在以后的生活中偶然发现。DAA的标准管理是手术修复。然而,建议对无症状或轻度病例进行观察和随访.手术修复与观察的长期结果没有得到很好的报道。我们描述了在我们机构进行手术修复与未修复的DAA患者的长期临床结果。方法检索18岁前诊断为DAA的患者的电子病历。来自临床的数据，放射学,和支气管镜检查结果，肺功能试验(PFT),提取心肺运动试验(CPET)。还对患者父母进行了关于过去和当前症状的结构化电话问卷。结果共纳入12例DAA患者(男8例,女4例)。中位年龄为8.5（1.5-17）岁。诊断时的年龄为60（1-192）个月。诊断后随访20(2-156)个月。五名病人接受了手术修复,七个病人没有修复。修复患者的手术年龄中位数为5（1-15）岁。电话问卷仅在10名患者中完成（5名修复和5名未修复）。在所有修复和未修复的患者中报告了婴儿期的呼吸道症状，并且在所有五名修复的患者和五名未修复的患者中的四名中都得到了解决。一名未修复的患者在劳累时抱怨间歇性呼吸困难。3名修复和3名未修复患者在婴儿期出现胃肠道症状，2名修复和1名未修复患者的胃肠道症状得到改善。在五名患者中进行了PFT（一名修复，四个未修复）并在一秒钟内显示正常的用力呼气量（FEV1），强迫肺活量(FVC)，所有患者的FEV1/FVC。在修复的患者和三名未修复的患者中观察到低峰值呼气流量（PEF）。在4名未修复的患者中进行了CPET，显示最大耗氧量（VO2-max）为预测的66％（58-88），最大通气量（VE-max）为75%（70-104），预测的通气储备为55%(48-104)。结论修复和未修复的DAA患者的长期临床预后均良好，即使两组均在婴儿期出现呼吸道症状。因此,临床观察是某些DAA患者的合法选择.
