Right pulmonary agenesis converts an incomplete vascular ring into a near-complete vascular ring.

A unique combination of pulmonary agenesis and anatomic left aortic arch with aberrant right subclavian artery was identified in two patients. Because of the right pulmonary agenesis, there is rotation of mediastinal contents, especially the aortic arch, into the right chest, converting an incomplete vascular ring to a near-complete vascular ring. The anterior portion of the ring is formed by the ascending aorta and aortic arch, whereas the posterior portion is formed by the aberrant right subclavian artery. Subclavian artery reimplantation and aortopexy effectively relieve the anterior and posterior tracheoesophageal compressive forces.