Thirty-three-year-old man with circumflex aorta and 4-cm right subclavian artery aneurysm.

A circumflex aorta is a form of vascular ring anomaly in which the aortic arch takes a retroesophageal pathway as it crosses to the contralateral side to become the descending thoracic aorta. The anomaly can cause a wide range of symptoms, ranging from asymptomatic to symptoms of respiratory distress or feeding difficulty due to tracheal and esophageal compression., Symptomatic patients can be treated using a multitude of methods, depending on their age and the exact congenital anomaly.

We present the case of a 33-year-old man with a history of CHARGE (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear abnormalities) syndrome, a bicuspid aortic valve, an atrial septal defect, and a double aortic arch. At 2 years old, he had had airway compromise secondary to compression of his trachea from the double aortic arch. He underwent left thoracotomy with division of the nondominant left-sided arch. Thirty years later, he had presented to our institution with recurrent upper respiratory tract infections and chronic dysphagia. Computed tomography angiography of his chest revealed a circumflex aorta with a right-sided aortic arch with a retroesophageal course as it transitioned to a left-sided descending thoracic aorta, which had caused displacement and compression of his trachea and esophagus. He was also found to have a 4-cm right subclavian artery aneurysm, with the dominant right vertebral artery originating from the aneurysm and an aberrant left subclavian artery creating a complete vascular ring. Our video describes our surgical approach to his circumflex aorta and right subclavian artery aneurysm with division of the retroesophageal component and reconstruction of the arch and right subclavian vertebral arteries through a median sternotomy with right supraclavicular extension and deep hypothermic circulatory arrest.