Computed tomography (CT) or magnetic resonance imaging (MRI) show the relationship of the aortic arches to the trachea and esophagus and also the degree of tracheal narrowing.
In the current era the risk of mortality or significant morbidity after surgical division of the lesser arch is low.
Recurrent respiratory infections are common and secondary pulmonary secretions can further increase the airway obstruction.
[5] Scheduled repair soon after birth in symptomatic patients can relieve tracheal compression early and therefore potentially prevent the development of severe tracheomalacia.
Barium swallow (esophagraphy): Historically the esophagram used to be the gold standard for diagnosis of double aortic arch.
In patients with double aortic arch the esophagus shows left- and right-sided indentations from the vascular compression.
Due to the blood-pressure related movement of the aorta and the two arches, moving images of the barium-filled esophagus can demonstrate the typical pulsatile nature of the obstruction.
Non-perfused elements of other types of vascular rings (e.g. left arch with atretic (closed) end) or the ligamentum arteriosum might be difficult to visualize by echocardiography.
The right aortic arch is completing the vascular ring by passing to the right and then behind the esophagus and trachea to join the usually left-sided descending aorta.
Surgical correction is indicated in all double aortic arch patients with obstructive symptoms (stridor, wheezing, pulmonary infections, poor feeding with choking).
It is useful to place pulse oximeter probes on both hands and one foot so that test occlusion of one arch or its branches will allow confirmation of the anatomy.
[citation needed] Isolated double aortic arch without associated intracardiac defects is a vascular anomaly that can be corrected without the support of cardiopulmonary bypass.
The end of the left arch is now further dissected from the mediastinal tissues for relief of any remaining constricting mechanism.
Additional relief can be obtained by stitching the lateral wall of the aorta to the adjacent rib to pull it away from the esophagus.
[citation needed] After insertion of a chest tube to prevent hemothorax and/or pneumothorax, the fourth and fifth rib are approximated by an absorbable suture.
[citation needed] A specific risk of open surgical repair of double aortic arch is injury to the recurrent laryngeal nerve, which can cause vocal cord paralysis.
Additional risks include lung injury, bleeding with the need for blood transfusions and wound infection.
[6] In 1837 von Siebold published a case report in the German medical literature entitled "Ringfoermiger Aortenbogen bei einem neugeborenen blausuechtigen Kinde" (Ring-shaped aortic arch in a cyanotic neonate).
[8] Gross is one of the pioneers of cardiovascular surgery, who also performed the first successful ligation of a patent ductus arteriosus 7 years earlier.
The basis for the radiologic diagnosis by barium swallow (esophagram) of double aortic arch (and other forms of vascular rings) was described in 1946 by Neuhauser from the same institution.