A double-barrelled aorta with high aortic Arch

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.