Situs Inversus Totalis and corrected transposition of the great arteries {I,D,D} in association with a previously unreported vascular ring

A 3-month-old girl with "noisy breathing" was found to have situs inversus totalis, corrected transposition of the great arteries {I,D,D}, and a vascu lar ring. The ring was composed of a left aortic arch with normal branching pattern and a right ligamentum arteriosum that extended from a diverticulu m off the descending aorta and coursed retroesophageal and to the right to join the pulmonary artery. There was no circumflex component of the aorta o r aberrant subclavian artery. The descending aorta was left sided. Compress ion of the esophagus and trachea was noted on contrast esophagram, magnetic resonance imaging (MRI), and at the time of surgery to divide the vascular ring. In association with her corrected transposition, the patient also wa s shown to have a mild Ebstein's deformity of the right-sided (systemic) at rioventricular valve and electrocardiographic evidence of Wolfe-Parkinson-W hite syndrome. The combination of situs inversus totalis, corrected transpo sition of the great arteries {I,D,D}, and an aortic arch anomaly has not be en previously reported. In addition, the aortic arch anomaly suggested by M RI imaging and confirmed at surgery has previously only been postulated to exist but to our knowledge never reported.