A 3-year-old girl with a history of respiratory distress was referred to emergency department. The patient was afebrile on initial evaluation, with a heart rate of 110, blood pressure of 75/40 mmHg, and oxygen saturation of 99% on room air. No wheezing or crackles were present on lung auscultation, and electrocardiography showed normal sinus rhythm. Chest X-ray showed tracheal indentation and raised the suspicion of tracheal stenosis.. Echocardiography was inconclusive but suggested the possibility of a double aortic arch. She was therefore referred for cardiac enhanced CT, which revealed a double aortic arch with arch origins for left common carotid artery, left vertebral artery, and left subclavian artery on the left; and right common carotid artery, right vertebral artery, and right subclavian artery on the right (Figure 1A and B). The patient subsequently underwent division of the left arch, distal to the origin of the left subclavian artery, to relieve the vascular compression on the trachea. Postoperative recovery was uneventful.

Figure 1.

Three-dimensional contrast-enhanced CT: (a) left sagittal view, showing the left common carotid artery, left vertebral artery, and left subclavian artery on the left aortic arch, as well as the (b) right sagittal view, showing right common carotid artery, right vertebral artery, and right subclavian artery on the right aortic arch.

Double aortic arch is an exceedingly rare congenital vascular anomaly, with one in 15,000 births accounting for only 1% of all CHDs. ^{Reference Bartsota, Jowett and Manuel1} It represents the most common form of a complete vascular ring, which can compress the trachea and/or oesophagus, leading to symptoms such as dysphagia, vomiting, respiratory distress, wheezing, and stridor. ^{Reference Biermann and Hazekamp2} Vertebral artery typically emanates from the supero-posterior aspect of the 1st part of the subclavian artery, and variations in the vertebral artery origin usually occur in the CHD. ^{Reference Lazaridis, Piagkou and Loukas3} In this case, enhanced CT revealed a rare vascular configuration with six arteries directly arising from the double aortic arch—an extremely unusual pattern not previously reported. To our knowledge, this is the first documented case of a double aortic arch in which both the right and left vertebral arteries originate directly from the respective aortic arches.

A more comprehensive understanding of such vascular variations is essential not only for accurate diagnosis but also for surgical planning. Prior studies have reported variable branching patterns of the aortic arch in both paediatric and adult patients with double aortic arch, including cases with isolated origins of carotid or subclavian arteries, and even aberrant vertebral artery origins. ^{Reference Bartsota, Jowett and Manuel1,Reference Lazaridis, Piagkou and Loukas3} However, dual direct vertebral artery origin from double arches remains undocumented in the literature to date.

This case underscores the importance of using enhanced CT imaging in evaluating patients with suspected double aortic arch, as it provides detailed vascular mapping, aiding in identifying rare branching anomalies. Furthermore, it offers embryological insights into the development and persistence of vascular segments during foetal life.