Coarctation of a right aortic arch

Right-sided aortic arch with a concomitant coarctation is an exceedingly rare congenital cardiac anomaly. We report of a 4-year-old boy who presented with a history of a stenotic bicuspid aortic valve who upon further evaluation was found to have a coarctation of a right-sided aortic arch. The frequency with which other anomalies exist in either of the above conditions requires thorough cardiac evaluation and detailed imaging. Surgical repair of this anomaly can safely be undertaken through a right thoracotomy.