Double Aortic Arch: A Case Series and Multidisciplinary Surgical Experience

Abstract

Background: Double aortic arch (DAA) is a rare congenital anomaly and the most common cause of a complete vascular ring. Although symptoms typically manifest in infancy, diagnosis may be delayed when clinical presentation is subtle or attributed to more common respiratory conditions.

Case summary: We report 3 patients with DAA who presented beyond infancy with long-standing, recurrent respiratory symptoms and feeding difficulties. In all cases, symptoms had been present for a prolonged period and were initially managed as recurrent respiratory or airway disease, resulting in delayed diagnosis. Imaging confirmed DAA with a right-dominant double aortic arch forming a complete vascular ring in each case. Surgical repair was performed via left thoracotomy, with posterior aortopexy required in 1 patient. All patients demonstrated marked postoperative clinical improvement.

Discussion: Delayed presentation of DAA can occur when symptoms are nonspecific or intermittently progressive, leading to misdiagnosis or late referral. DAA should therefore be considered in children with persistent or recurrent respiratory or gastrointestinal symptoms unresponsive to standard therapy. Surgical correction via left thoracotomy is safe and effective, and results in substantial symptomatic relief with low morbidity.

Take-home messages: DAA may present late due to nonspecific symptoms and diagnostic delay. Heightened clinical awareness, appropriate cross-sectional imaging, and timely surgical intervention are essential to prevent prolonged morbidity and achieve excellent outcomes.

Keywords: aorta; computed tomography; congenital heart defect; imaging; pediatric surgery; postoperative; thoracotomy; treatment; ultrasound.