Congenital Heart Disease. 2017 Sep; 12(5):583-587

DOI 10.1111/chd.12487

Received: 25 April 2017 / Accepted: 11 May 2017

Epub 2017 Jun 5

© 2017 Wiley Periodicals, Inc.


Right aortic arch with situs solitus frequently heralds a vascular ring


William N Evans, MD, Ruben J Acherman, MD, Michael L Ciccolo, MD, Sergio A Carrillo, MD, Gary A Mayman, MD, Carlos f Luna, MD, Robert C Rollins, MD, William J Castillo, MD, Alvaro Galindo, MD, Abraham Rothman, MD, John A Alexander, MD, Tina W Kwan, MD, Humberto Restrepo, MD, MPH



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




Objective: We hypothesized that a right aortic arch in situs solitus, with or without an associated cardiovascular malformation, is often associated with a vascular ring.


Methods: From those born in Southern Nevada between March 2012 and March 2017, we identified 50 (3.6 per 10,000 live births) with a right aortic arch and situs solitus. From the 50 patients, 6 did not meet inclusion criteria for further analysis.


Results: Of the 44 remaining, 33 (75%) had a vascular ring. Of the 33 with a vascular ring, 26 (79%) occurred with an isolated right aortic arch, and 7 (21%) had an associated cardiovascular malformation. Of the total 44 patients with a right aortic arch in situs solitus, 34 (79%) were diagnosed prenatally.


Conclusions: In conclusion, we found a right aortic arch in situs solitus was often associated with a vascular ring. Further, to the best of our knowledge, no previous general population study has demonstrated an equal or higher right aortic arch, prenatal detection rate of 79%.


Full Article Text