A 65-year-old previously asymptomatic woman with a history of tobacco use presented after experiencing an hour of chest pain and syncope. A 12-lead electrocardiogram revealed an inferior ST-elevation myocardial infarction. Coronary angiography revealed an acute thrombotic occlusion of the right coronary artery (Fig. 1); however, there was difficulty in maneuvering diagnostic catheters to the coronary ostia. Subsequent aortography revealed a double aortic arch (Fig. 2). After revascularization, 64-slice computed tomography (CT) of the aorta (Fig. 3) showed a dominant right aortic arch (Fig. 4A) to the right of the trachea, with separate ostia of the right subclavian and common carotid arteries. A more inferiorly located left aortic arch (Fig. 4B) connected the ascending and descending aorta at the left of the trachea, with separate ostia of the left subclavian and common carotid arteries. No vascular ring was seen. The aorta was also right sided (Fig. 4C).

Fig. 1.

Coronary angiogram, left anterior oblique view, shows an acute thrombotic occlusion (arrow) of the right coronary artery.

Fig. 1.

Coronary angiogram, left anterior oblique view, shows an acute thrombotic occlusion (arrow) of the right coronary artery.

Fig. 2.

Aortogram, left anterior oblique view, shows evidence of a double aortic arch system.

AA = ascending aorta; DA = descending aorta; L = left aortic arch; LCCA = left common carotid artery; LSA = left subclavian artery; R = right aortic arch; RCCA = right common carotid artery; RSA = right subclavian artery

Fig. 2.

Aortogram, left anterior oblique view, shows evidence of a double aortic arch system.

AA = ascending aorta; DA = descending aorta; L = left aortic arch; LCCA = left common carotid artery; LSA = left subclavian artery; R = right aortic arch; RCCA = right common carotid artery; RSA = right subclavian artery

Fig. 3.

This 64-slice cardiac computed tomogram is a 3-dimensional volume rendering (left anterior oblique view) that correlates with the aortographic view in Fig. 2.

AA = ascending aorta; DA = descending aorta; L = left aortic arch; LCCA = left common carotid artery; LSA = left subclavian artery; MPA = main pulmonary artery; R = right aortic arch; RCCA = right common carotid artery; RSA = right subclavian artery

Fig. 3.

This 64-slice cardiac computed tomogram is a 3-dimensional volume rendering (left anterior oblique view) that correlates with the aortographic view in Fig. 2.

AA = ascending aorta; DA = descending aorta; L = left aortic arch; LCCA = left common carotid artery; LSA = left subclavian artery; MPA = main pulmonary artery; R = right aortic arch; RCCA = right common carotid artery; RSA = right subclavian artery

Fig. 4.

64-Slice cardiac computed tomograms (all axial views) at the levels of the A) right and B) left aortic arches and the C) aortic valve. In C, note the stent (arrow) in the right coronary artery at the level of the aortic valve. The descending aorta is right sided.

AA = ascending aorta; Ao = aortic valve; DA = descending aorta; E = esophagus; L = left aortic arch; LA = left atrium; LCCA = left common carotid artery; LSA = left subclavian artery; R = right aortic arch; RA = right atrium; RCA = right coronary artery; RVOT = right ventricular outflow tract; T = trachea

Fig. 4.

64-Slice cardiac computed tomograms (all axial views) at the levels of the A) right and B) left aortic arches and the C) aortic valve. In C, note the stent (arrow) in the right coronary artery at the level of the aortic valve. The descending aorta is right sided.

AA = ascending aorta; Ao = aortic valve; DA = descending aorta; E = esophagus; L = left aortic arch; LA = left atrium; LCCA = left common carotid artery; LSA = left subclavian artery; R = right aortic arch; RA = right atrium; RCA = right coronary artery; RVOT = right ventricular outflow tract; T = trachea

Comment

A double aortic arch is a variant of vascular ring, occurring in 0.05% to 0.3% of the general population.1  It can encircle the trachea and esophagus via connected segments of aortic arch, causing symptoms of dyspnea, stridor, or dysphagia in infancy or early childhood2,3 ; rarely, these symptoms develop in adulthood.4–,6  Double aortic arch is the result of persistence of both the left and right embryonic 4th arches and dorsal aortas.7  Aortic arch “sidedness” is the side of the trachea that the arch passes as it crosses a mainstem bronchus.8  Cardiovascular CT can provide definitive evaluation of the course and orientation of these aortic arch variants and of associated cardiac anomalies. This case illustrates the supplementary role of CT with invasive aortography in diagnosing a rare aortic anatomic variant.

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Author notes

Section Editor: Raymond F. Stainback, MD, Department of Adult Cardiology, Texas Heart Institute, 6624 Fannin St., Suite 2480, Houston, TX 77030

From: Division of Cardiology, Department of Medicine (Drs. Akhondi, Tabibiazar, and Yang) and Cardiovascular Imaging Section, Department of Radiology (Dr. Ruehm), University of California at Los Angeles, Los Angeles, California 90095