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Page 12 of 14 Bader et al. Vessel Plus 2020;4:34 I http://dx.doi.org/10.20517/2574-1209.2020.36

Post-operative issues
Due to its proximity, recurrent laryngeal nerve injury is a reported post-operative complication especially
when a more extensive procedure such as aortic uncrossing surgery is required [4,14] . We had not encountered
[6,7]
this complication in our reported surgical experience in both circumflex right and left aortic arch . Other
complications associated with arch surgery include phrenic nerve injury and chylothorax.

Persistent respiratory symptoms can be due to (1) residual compression from retroesophageal arch if only
arterial ligament division alone was performed; and/or (2) underlying tracheobronchomalacia. In children
who presented early in the neonatal period or infancy, underlying tracheomalacia could be severe and
[7]
persistent even after total anatomical correction . Failure to extubate will require tracheostomy and a home
ventilator may be required. Posterior tracheobronchopexy has been used following repair of hypoplastic
circumflex aortic arch to facilitate extubation and avoid tracheostomy and long-term ventilation .
[7]
Concomitant circumflex aortic arch repair with posterior tracheopexy has been reported recently by the
[35]
Boston group .

CONCLUSION
The role of surgical intervention in the management of circumflex aorta is evolving. The prevalence of
functionally circumflex aorta and persistent symptoms following double aortic arch repair remains to be
defined. The aortic arch uncrossing procedure provides full anatomical correction for circumflex aorta
with right aortic arch, but requires hypothermic circulatory arrest, and therefore considerable debate still
exists if this is always required in the absence of arch obstruction. Undoubtedly, the aortic arch uncrossing
procedure has an important role as a rescue strategy in patients with significant residual symptoms post-
operatively but its role as a primary strategy is also emerging for severely symptomatic patients, especially
with improvised technique to minimize operative morbidities. Posterior aortic translocation also provides
full anatomical correction in circumflex variants with appropriate anatomy, without the need of arch
uncrossing. When arch obstruction is present, full anatomical repair with aortic uncrossing or posterior
aortic translocation should always be advocated to relieve arch obstruction as well as to correct the
pathological effects of circumflex aorta.

DECLARATIONS
Acknowledgments
The authors are extremely grateful to Miss Mhari Chambers, RGN for all her drawings in this manuscript.

Authors’ contributions
Made substantial contributions to conception and design of the study, performed literature review and
preparation of the manuscript: Bader V, Peng E
Made substantial contributions to conception and design of the study and manuscript review: Knight WB,
Danton MH

Availability of data and materials
Not applicable.

Financial support and sponsorship
None.

Conflicts of interest
All authors declared that there are no conflicts of interest.

Ethical approval and consent to participate
Not applicable.