Stridor serving as a harbinger of the mirror-image right aortic arch carrying three anatomic dispositions contributing to a noosing vascular ring

Özet

Right aortic arch (RAA) is a rare congenital aortic arch anomaly, accounting for 1 out of 1,000 individuals in radiographic and autopsy studies (1). RAA was classified by Edwards into three types: RAA with mirror-image branching (MIRAA) (type III A), RAA with aberrant left subclavian artery (type III B) (with or without Kommerell diverticulum), and RAA with aberrant left brachiocephalic artery and isolated left subclavian artery (type III C) (2). MIRAA, which is the second most common type of RAA, results from evolutional regression changes of the left dorsal aorta distal to the seventh intersegmental artery, regression of the left sixth arch, and persistence of the right sixth arch (2). MIRAA was frequently associated with concomitant congenital heart disease in pediatric patients, especially those with tetralogy of Fallot, pulmonary atresia, and truncus arteriosus (1, 3). We reported on a 4-month-old boy who had a complete vascular ring (VR), composed of an “isolated” MIRAA (III A RAA), a right-sided retroesophageal aortic diverticulum (AD), and a ligamentum arteriosum arising from the AD to the left pulmonary artery (LPA). The anatomic dispositions that are liable to form a complete VR will be discussed.