Vascular ring: left cervical aortic arch, right descending aorta, and right ligamentum arteriosum

A rare form of vascular ring is presented consisting of a left cervical aortic arch, a right descending aorta, and a right ligamentum arteriosum in a symptomatic infant. The planning of its surgical interruption was complicated by absence of the left pulmonary artery and hypoplasia of the left lung. This case provides an exception to the surgical axiom that all vascular rings can be divided through the left side of the chest. It reveals the difficulty in evaluation of these lesions by barium swallow alone and emphasizes the importance of preoperative angiography to correctly define the anatomy. The surgical approach to a patient with an absent left pulmonary artery and hypoplastic lung requiring a contralateral thoracotomy is presented.