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Double aortic arch surgery11/25/2023 Vascular rings and pulmonary artery sling. Vascular rings: 10-year review of imaging. Backer CL, Mavroudis C, Rigsby CK, Holinger LD.Mediastinal vascular anomalies causing tracheal and esophageal compression and obstruction in childhood. Surgical management of congenital vascular rings. Arciniegas E, Hakimi M, Hertzler JH, Farooki ZQ, Green EW. The aorta starts normally at the heart as one large vessel, but then divides into two arches: one on each side of the trachea and esophagus.Smith BM, Lu JC, Dorfman AL, Mahani MG, Agarwal PP.Surgical relief for tracheal obstruction from a vascular ring. The diagnosis of vascular ring can be challenging after ductal closure and arch atresia occurs. To conclude, symptomatic patients should be highly suspicious of vascular ring. This occurs with double aortic arches and configurations with aberrant subclavian arteries. The sign is present when the two dorsal subclavian arteries arise directly from the aortic arch and notfrom a brachiocephalic artery. One clue to an arch anomaly is the “four artery sign”which can be seen on sections obtained cephalad to the aortic arch and consists of two dorsal subclavian arteries and two ventral carotid arteries evenly spaced around the trachea. In these cases the diagnosis of a complete ring depends on recognizing the arterial branching pattern, the side of the aortic arch, and focal narrowing of the airway. However, in some cases the imaging diagnosis by echocardiography (ECHO), CTA or Magnetic Resonance Imaging (MRI) is difficult due to the presence of the atretic segment. Computed Tomography Angiogram (CTA) is very accurate in the identification of vascular anomalies of the aortic arch and great vessels. The symptoms typically include respiratory distress, stridor, the classic “seal bark” cough, apnea, dysphagia, and recurrent respiratory tract infections. Most children with vascular rings present with symptoms within the first several weeks to months of life. Symptoms of vascular ring relate to the consequences of tracheal and esophageal compression. Both arches can be equally patent (5%), there is hypoplasia or atresia of the left arch (70%–75%), or the right arch (20%). Double aortic arch accounts for 50% of vascular rings. Vascular rings of aortic arch origin account for 1% to 2% of cases of congenital heart disease. Vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the trachea or esophagus or both.
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