Cong Heart Disease 2018 Jul;13(4):624-627

DOI 10.1111/chd.12623

[Epub ahead of print].

Received: 19 March 2018 / Revised: 1 May 2018 / Accepted: 2 May 2018

© 2018 2018Wiley Periodicals, Inc.


Right aortic arch with situs solitus



William N Evans, MD, Ruben J Acherman, MD, Dean Berthoty, MD, Gary A Mayman, MD, Michael M Ciccolo, MD, Sergio A Carrillo, MD, Humberto Restrepo, MD, MPH



Children’s Heart Center Nevada, 3006 S Maryland Pkwy, Ste 690, Las Vegas, Nevada 89109, USA. Email:



Patients: We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right aortic arch that were born between January 2000 and January 2018. Results: From our databases, we identified 204 patients. We excluded patients with a double aortic arch from analysis. Of the 204 patients, 103 (50%) were male. Of the 204 patients, 95 (47%) had an isolated right aortic arch.Of the 95 with an isolated right aortic arch, 4 (4%) had chromosome 22q11.2 deletion syndrome, and 89 (94%) had a vascular ring. Of the 204 patients, 109 (53%) had a surgical intracardiac malformation. Of the 109, with an associated surgical intracardiac malformation, 38 (35%) had chromosome 22q11.2 deletion syndrome, and 28 (26%) had a vascular ring. Overall, of the 204 patients, 200 (98%) had a vascular ring, an associated intracardiac malformation, or both. Prenatal detection was 85% (40/47) for the subset of patients born in Southern Nevada between January 2015 and January 2018. Conclusion: this review, to best of our knowledge, reports one of largest series of right aortic arches in the literature. Data from our cohort demonstrates that a right aortic arch in situs solitus is almost always associated with pathology. Further, our center’s right aortic arch prenatal detection rate exceeds previous reports.


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