Double aortic arch, tetralogy of Fallot with pulmonary atresia and atrioventricular septal defect

A double aortic arch is usually an isolated abnormality. We describe a case with a previously undescribed combination of tetralogy of Fallot with pulmonary atresia, complete atrioventricular septal defect, and left patent arterial duct in association with a double aortic arch. A complete diagnosis was made by echocardiography. Meticulous suprasternal echocardiography must be employed to avoid overlooking an unsuspected aortic arch abnormality.