Double aortic arch
Citation, DOI & article data
Double aortic arch is mostly diagnosed in childhood due to symptoms related to esophageal and/or tracheal obstruction. Respiratory symptoms can be more common in infancy or early childhood while adult patients complain of difficulties in swallowing rather than respiratory difficulties due to tracheal development.
This anomaly is caused by persistence of the right and left embryonic fourth aortic arches, which results in formation of a vascular ring from the splitting of the ascending aorta into two limbs that pass to either side of the trachea and esophagus 5 (both of which get encircled), which then join as a single descending aorta.
When the minor arch is markedly hypoplastic or atretic (incomplete double aortic arch), the affected segment tends to be almost always distal to the left subclavian artery 9.
- right dominant arch: ~75-80%
- codominant arch: ~5%
- left dominant arch: ~25%
It is rarely associated with congenital heart disease 12,13.
Right-sided aortic arch indenting the trachea and increase right paratracheal soft tissue thickness. Lack of air column in the thoracic portion of the trachea is sometimes seen.
Fluoroscopy: barium swallow
Contrast swallow studies are more helpful and demonstrate:
- classic "reverse S" indentation of the contrast column on frontal view produced by an upper indentation of the right-sided aortic arch and the lower indentation is by the left-sided aortic arch
- posterior indentation on lateral view produced by the encircling posterior arch (mostly right-sided aortic arch component)
CT and MRI
Contrast-enhanced cross-sectional imaging is required to confirm the diagnosis and also to delineate details of anatomy in particular right or left arch dominance for surgical planning.
Treatment and prognosis
Surgical intervention with tracheostomy from the side of descending aorta and division of the minor (smaller) arch and ligamentum arteriosum is indicated for symptomatic patients. In appropriate cases, surgical planning is directed toward transection of either the smallest arch (if both arches are patent) or an atretic arch segment.
- 1. Brockmeier K, Demirakca S, Metzner R et-al. Images in cardiovascular medicine. Double aortic arch. Circulation. 2000;102 (12): E93-4. Circulation (full text) - doi:10.1161/01.CIR.102.12.e93 - Pubmed citation
- 2. Berdon WE. Rings, slings, and other things: vascular compression of the infant trachea updated from the midcentury to the millennium-the legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD. Radiology. 2000;216 (3): 624-32. doi:10.1148/radiology.216.3.r00se40624 - Pubmed citation
- 3. Ito H, Konishi A, Kon-Nai T et-al. Double aortic arch with atresia, tapering and aneurysm of the left arch. Br J Radiol. 2006;79 (944): e71-4. doi:10.1259/bjr/28729845 - Pubmed citation
- 4. Ikenouchi H, Tabei F, Itoh N et-al. Images in cardiovascular medicine. Silent double aortic arch found in an elderly man. Circulation. 2006;114 (8): e360-1. Circulation (full text) - doi:10.1161/CIRCULATIONAHA.106.617274 - Pubmed citation
- 5. Noguchi K, Hori D, Nomura Y et-al. Double aortic arch in an adult. Interact Cardiovasc Thorac Surg. 2012;14 (6): 900-2. doi:10.1093/icvts/ivs060 - Free text at pubmed - Pubmed citation
- 6. Koch AM, Glöckler M, Cesnjevar R. Double aortic arch. Eur. Heart J. 2013;34 (20): 1516. doi:10.1093/eurheartj/ehs268 - Pubmed citation
- 7. Kellenberger CJ. Aortic arch malformations. Pediatr Radiol. 2010;40 (6): 876-84. doi:10.1007/s00247-010-1607-9 - Pubmed citation
- 8. Satyapal KS, Lazarus L, Shama D. Double aortic arch: an unusual congenital variation. Surg Radiol Anat. 2013;35 (2): 125-9. doi:10.1007/s00276-012-1030-z - Pubmed citation
- 9. Ramos-Duran L, Nance JW, Schoepf UJ et-al. Developmental aortic arch anomalies in infants and children assessed with CT angiography. AJR Am J Roentgenol. 2012;198 (5): W466-74. doi:10.2214/AJR.11.6982 - Pubmed citation
- 10. Kimura-Hayama ET, MeléNdez G, MendizáBal AL et-al. Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 2010;30 (1): 79-98. Radiographics (full text) - doi:10.1148/rg.301095061 - Pubmed citation
- 11. Haramati LB, Glickstein JS, Issenberg HJ et-al. MR imaging and CT of vascular anomalies and connections in patients with congenital heart disease: significance in surgical planning. Radiographics. 2002;22 (2): 337-47. doi:10.1148/radiographics.22.2.g02mr09337 - Pubmed citation
- 12. Türkvatan A, Büyükbayraktar FG, Olçer T et-al. Congenital anomalies of the aortic arch: evaluation with the use of multidetector computed tomography. Korean J Radiol. 2009;10 (2): 176-84. doi:10.3348/kjr.2009.10.2.176 - Free text at pubmed - Pubmed citation
- 13. Kau T, Sinzig M, Gasser J et-al. Aortic development and anomalies. Semin Intervent Radiol. 2007;24 (02): 141-52. doi:10.1055/s-2007-980040 - Free text at pubmed - Pubmed citation
- 14. Hanneman K, Newman B, Chan F. Congenital Variants and Anomalies of the Aortic Arch. Radiographics : a review publication of the Radiological Society of North America, Inc. 37 (1): 32-51. doi:10.1148/rg.2017160033 - Pubmed
- 15. Priya S, Thomas R, Nagpal P, Sharma A, Steigner M. Congenital anomalies of the aortic arch. (2018) Cardiovascular diagnosis and therapy. 8 (Suppl 1): S26-S44. doi:10.21037/cdt.2017.10.15 - Pubmed