[Repair of D-transposition of the great arteries associated with double aortic arch]

We report a case of D-transposition of the great arteries associated with double aortic arch. An aortic root angiogram revealed that the left-sided aortic arch was anterior and smaller than the right one and the descending thoracic aorta located on the left side of the spine. Balloon atrial septostomy was performed at one day of age. The patient underwent a simultaneous arterial switch procedure and division of the vascular ring at the isthmus of the left aortic arch through a median sternotomy incision at the age of 16 days. There was persistent postoperative difficulty in weaning the patient from the ventilator. Magnetic resonance images showed re-formation of pseudovascular ring by the connective tissue grown around the divided arch. At the age of 23 days, resection of the remnant of left aortic arch including the left subclavian artery and the diverticulum concomitant with vascular suspension procedure was performed through a left lateral thoracotomy. The patient was subsequently extubated without difficulty and was discharged from the hospital. It is though that a vascular suspension procedure and resection of the subclavian artery are necessary to avoid respiratory obstruction when the great arteries are in an anteroposterior position and Lecompte procedure is performed.