Open Access

ARTICLE

Right aortic arch with situs solitus

William N. Evans^1,2, Ruben J. Acherman^1,2, Dean Berthoty³, Gary A. Mayman^1,2, Michael L. Ciccolo^1,4, Sergio A. Carrillo^1,4, Humberto Restrepo^1,2

1 Children’s Heart Center Nevada, Las Vegas, Nevada, USA
2 Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada Las Vegas, School of Medicine, Las Vegas, Nevada, USA
3 Red Rock Radiology, Las Vegas, Nevada, USA
4 Department of Surgery, University of Nevada Las Vegas, School of Medicine, Las Vegas, Nevada, USA

* Corresponding Author: William N Evans, MD, Children’s Heart Center Nevada, 3006 S Maryland Parkway Ste 690, Las Vegas, NV 89109. Email:

Congenital Heart Disease 2018, 13(4), 624-627. https://doi.org/10.1111/chd.12623

Abstract

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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Keywords

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