A double-barrelled aorta with high aortic Arch

Abstract

A double-barrelled aorta was detected in a female newborn with 22q11.2 deletion syndrome. Double-barrelled aorta had been previously described as persistence of the fifth pharyngeal arch, but its existence continues to be debated. Recent embryologic studies suggest that double-barrelled aorta is more likely explained by other developing processes in the majority of cases. In our case, catheter angiography confirmed the presence of the high aortic arch and double-barrelled aorta. The upper lumen was located above the level of the clavicles. These findings suggested that the persistence of the segment of dorsal aorta between the third and fourth embryonic arches and the double-barrelled aorta was more likely a consequence of persistence of the third and fourth pharyngeal arches. Detailed imaging and embryologic considerations played an important role in accurate assessment of the origin of the double-barrelled aorta. <Learning objective: Double-barrelled aorta is a rare congenital anomaly. Its etiology had been previously described as persistence of the fifth pharyngeal arch, but recent embryologic studies suggest that it is more likely explained by other several developing processes. We report a case of double-barrelled aorta with high aortic arch likely arose from the third and fourth pharyngeal arches. Although the therapeutic approaches remain the same, accurate description and classification are important.>.

Keywords: 22q11.2 deletion; Double-barrelled aorta; Double-lumen aorta; High aortic arch; Persistent fifth aortic arch.

Fig. 1

Two-dimensional echocardiography at birth (a) and three-dimensional reconstruction of computed tomography angiograms at Day 9 (b-d). (a) Suprasternal parasagittal view demonstrating a superior lumen (white triangle) giving rise to the head and neck vessels and an inferior lumen (white asterisk) with no branching extending from the ascending to the descending aorta underneath the transverse aortic arch. (b) Anterior view showing the right aortic arch and the aberrant origin of the left subclavian artery (white arrow). (c) Lateral view showing the superior arch (white arrow) and the inferior arch (white dotted arrow). (d) The superior arch (white arrow) was located above the level of the clavicle (white asterisk) forming a high aortic arch.

Fig. 2

Cardiac catheterization aortograms at Day 24 showing the brachiocephalic artery (1), right carotid artery (2), right subclavian artery (3), and left subclavian artery (4). (a) The right aortic arch and the aberrant origin of the left subclavian artery are demonstrated in this anterior view. The superior arch (white arrow) is located above the level of the clavicle (black asterisk). (b) In the lateral view, the inferior (black arrow) and superior arches (white arrow) can be seen branching from the ascending aorta unequivocally distal to the origin of the brachiocephalic artery and terminating directly in the dorsal aorta.