Double aortic arch associated with common inlet left ventricle.

A double aortic arch is usually an isolated abnormality. We report a case with a previously undescribed combination of double aortic arch and common inlet left ventricle. The patient underwent division of the nondominant right aortic arch and the right ductus arteriosus at 2 months of age following a left modified Blalock-Taussig shunt. At 3 years of age, extracardiac total cavopulmonary connection was successfully performed via bidirectional cavopulmonary anastomosis.