Diagnostic Considerations

Clinicians should promptly diagnose and properly define the anatomy of a double aortic arch.

Special concerns

There are no special issues or concerns related to pregnancy in patients with repaired vascular ring.

In patients with an unrepaired ring, pregnancy-induced physiologic changes should not be of special concern, although symptoms may be exacerbated in certain situations.

Differential Diagnoses

Asthma
Bronchiolitis
Congenital Stridor
Laryngeal Web
Laryngomalacia
Pediatric Subglottic Stenosis Surgery
Pulmonary Artery Sling
Respiratory Syncytial Virus Infection
Right Aortic Arch in Vascular Ring Defects
Stridor
Tracheomalacia

Workup

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Media Gallery

Double Aortic Arch. Schematic diagram (left) of the primitive pharyngeal arch system shows the left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries, fourth (IV) and sixth (VI) pharyngeal arches, distal pulmonary arterial segments (PA), dorsal aortas (DA), and seventh intersegmental arteries (VII). The proximal (p) sixth arches develop into the proximal pulmonary arteries and the distal (d) sixth arches become the arterial ducts. The seventh intersegmental arteries develop into the subclavian arteries. Schematic diagram (right) shows the segments of the pharyngeal arch system that regress (shown in black) in the normal formation of the thoracic great arteries. Left pulmonary artery (LPA); ductus arteriosus (PDA); right pulmonary artery (RPA); subclavian artery (SCA).
Double Aortic Arch. Schematic diagram (left) depicts the segments of the pharyngeal arch system that regress (shown in black) so that the mature vascular anatomy of a double aortic arch can develop. The dominant and minor arches can vary in laterality and specific patterns of branching and segmental hypoplasia/atresia. (These variables are not specified in this diagram.) Left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries; fourth (IV) and sixth (VI) pharyngeal arches; distal pulmonary arterial segments (PA); dorsal aortas (DA); seventh intersegmental arteries (VII); proximal (p) sixth arches; distal (d) sixth arches. Mature anatomy (right) of a double aortic arch with a dominant right arch and patent minor left arch. In most patients, a single left-sided ductus arteriosus or ligamentum arteriosum is present. Left pulmonary artery (LPA); ductus arteriosus (PDA); right pulmonary artery (RPA); subclavian artery (SCA).
Double Aortic Arch. Transverse MRI images in a patient with double aortic arch. Both arches are patent; the right arch is dominant. Images A-F are arranged in a caudad to cephalad order. (A) Transverse image at the level of the pulmonary valve. The ascending aorta (AAo) and descending aorta (DAo), cephalad to the junction of the left and right arches, can be seen. (B) At the level of the pulmonary artery (PA) bifurcation, the distal confluence of the left and right arches forming the single descending aorta is depicted. (C) The distal portions of the left (L) and right (R) arches can be seen posterior and to the left and right sides of the trachea. Note the anteroposterior compression of the tracheal carina (anterior to and between the arches). (D) Moving cephalad, the dominance of the right arch can be seen. (E) At the level of the proximal/transverse aortic arches, the origin of the left and right arches from the rightward ascending aorta can be seen. (F) The left and right common carotid and subclavian arteries arise from the left and right arches, respectively. The common carotid arteries are the dark round structures anterior to and to either side of the trachea. The subclavian arteries are the dark round structures posterior to and to either side of the trachea.
Double Aortic Arch. Coronal spin-echo MRI images in a patient with a double aortic arch. Both arches are patent, with the right (R) slightly larger in caliber than the left (L). Compression of the trachea (T) between the two arches can be seen (left). The confluence of the arches and the descending aorta (D) are shown (right).