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Page 10 of 14 Bader et al. Vessel Plus 2020;4:34 I http://dx.doi.org/10.20517/2574-1209.2020.36

Division of the arterial ligament alone, however, does not address compression of the mediastinal organs
from the retroesophageal arch segment. In the absence of aortic arch hypoplasia, this approach may be
justifiable in patients with mild symptoms or asymptomatic patients who underwent other cardiac or
[33]
thoracic procedures .
Aortic arch uncrossing procedure
Optimal symptomatic relief may not be achieved by dividing the arterial ligament alone due to persistent
retroesophageal aortic arch compression. Therefore, the aortic uncrossing procedure was described by the
French group in 1984 in three infants with circumflex aortic arch - all of whom had previously undergone
[1,2]
ligamentum division alone but remained ventilator dependent . These 3 patients were extubated after
undergoing aortic uncrossing procedure; and became the subjects on three French reports of circumflex
arch [1,2,14] .

The aortic uncrossing procedure is performed through a median sternotomy with cardiopulmonary
bypass, hypothermia, and a short period of circulatory arrest. Deep hypothermic total circulatory arrest
is used in the original and subsequent series [1,2,4] . The aorta is transected distal to the origin of the right
subclavian artery and the proximal stump is over-sewn. The ligamentum arteriosum is ligated and divided.
The retroesophageal aortic arch is dissected from its posterior attachments and brought anteriorly to the
left side of the ascending aorta. An arteriotomy is performed on the side of the ascending aorta below the
left carotid artery. An end to side anastomosis is performed. The right subclavian artery can be divided to
facilitate mobilization of the arch anteriorly, although this is not mandatory [4,14] . Selective antegrade cerebral
perfusion with continuous cardiac perfusion and moderate hypothermia - avoiding total body circulatory
[6]
arrest - is an alternative cardiopulmonary bypass strategy [Figure 8] .
Posterior aortic translocation
The surgical approach to address circumflex aorta with left aortic arch is less well described . The aortic
[7,9]
arch is on the left side, and therefore does not needs to be uncrossed to the “normal” side. In our own
experience and of past reports, the proximal descending aorta is on the right but later courses back to the
[7]
left . Therefore, instead of “uncrossing” the arch, posterior aortic translocation is required. The presence
of the right-sided location of the proximal descending aorta is the main issue, which requires translocation
into the left chest. The division of the ligamentum relieves the ring and the translocation procedure
removes the posterior airway compression. This procedure requires extensive mobilization of the entire
descending thoracic aorta. In the presence of unobstructed arch, posterior aortopexy is performed. The
procedure can be approached via left thoracotomy without cardiopulmonary bypass. In the presence of the
arch hypoplasia, the retroesophageal segment can be resected and an extended end-to-end anastomosis can
[7]
be performed [Figure 9] .
Aortic arch hypoplasia or obstruction
Several surgical approaches have been reported for the management of circumflex aorta with hypoplastic
arch. These include addressing the arch obstruction alone without correcting the circumflex anatomy -
resection of the coarctation, relieving the obstruction either using a patch or placing an extra anatomic
graft. However, this approach will leave a complex vascular ring in situ which could be a problematic issue
in the future with persistent oesophageal and airway compression.

Full anatomical correction of circumflex aorta will therefore require the following procedures: circumflex
aorta with hypoplastic right aortic arch: resection of the hypoplastic segment, aortic arch uncrossing
procedure and end-to-side anastomosis to the aorta anteriorly. Cardiopulmonary bypass with deep
hypothermic total circulatory arrest can be used; but we preferred the strategy of selective antegrade
[6]
cerebral perfusion via the innominate artery perfused via a 3 mm to 3.5 mm Goretex shunt . Aortic