[Successful staged repair of corrected transposition of great arteries and mitral atresia associated with double aortic arch].

We report a rare case of corrected transposition of great arteries and mitral atresia associated with double aoric arch. The patient showed polypnea and cyanosis soon after birth. She underwent balloon atrioseptostomy at the age of 18 days and pulmonary artery banding (PAB) at 24 days. But she showed severe persistent stridor after PAB. Esophagography and left counter-current aortography revealed double aortic arch. At the age of 49 days, division of the left lesser arch and ligation of patent ductus arteriosus were performed through left lateral thoracotomy. Stridor disappeared completely following operation. She underwent total cavopulmonary connection using a 18 mm Gore-Tex graft as intraatrial conduit concomitant with pacemaker implantation at the age of 8 years. Postoperative course was uneventful. To our knowledge, this is the first successful case report of surgical correction of corrected transposition of great arteries and mitral atresia associated with double aoric arch.