Additional relief can be obtained by stitching the lateral wall of the aorta to the adjacent rib to pull it away from the esophagus.After insertion of a chest tube to prevent hemothorax and/or pneumothorax, the fourth and fifth rib are approximated by an absorbable suture. The surgery is completed by closure of the left thoracotomy wound in layers.In most centers, the mortality risk for surgery is between zero and 2%. A specific risk of open surgical repair of double aortic arch is injury to the recurrent laryngeal nerve, which can cause vocal cord paralysis. Injury to the lymphatic system can lead to postoperative chylothorax. Additional risks include lung injury, bleeding with the need for blood transfusions and wound infection. Postoperative care
After the surgery, some patients require intubation and mechanical ventilation for several days to allow adequate tracheal toilet, but most patients can have the tubes removed soon after the surgery. The obstructive airway symptoms may be worse in the first postoperative weeks. Only a few patients have immediate relief of stridor, but many obtain immediate relief of problems with swallowing (dysphagia). After extubation, it might be necessary to maintain positive airway pressure by appropriate flows of a humidified oxygen/air mixture. Epidemiology
Complete vascular rings represent about 0.5-1% of all congenital cardiovascular malformations. The majority of these are double aortic arches. There is no known gender preference, i.e. males and females are about equally affected. There is also no known ethnic or geographic disposition.Associated cardiovascular anomalies are found in 10-15% of patients.
The smaller left arch passes anteriorly and to the left of the trachea in the usual position. It is often joined by the ligamentum arteriosum (or patent ductus arteriosus) where it forms the descending aorta. The left arch gives first origin to the left common carotid artery and then the left subclavian artery. 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. The first vessel coming off the right arch is usually the right common carotid artery followed by the right subclavian artery. Double aortic arch with left dominant arch:
In this less common condition, as the name indicates, the left arch is the larger of the two aortic arches. The origins of the arm and head vessels are identical to the anatomy of double aortic arch with right dominant arch
Balanced or codominant double aortic arch:
In this rare condition both aortic arches are of the same diameter. Treatment
Surgical correction is indicated in all double aortic arch patients with obstructive symptoms (stridor, wheezing, pulmonary infections, poor feeding with choking). If symptoms are absent a conservative approach (watchful waiting) can be reasonable. Children with very mild symptoms may outgrow their symptoms but need regular follow-up. Anesthesia and intraoperative monitoring
The procedure is performed in general anesthesia. 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.