Tetralogy of Fallot with double aortic arch: successful staged repair. Case report and review of literature.

The rare association of DAA with TOF is reported in a young boy who underwent successfully at the age of 3 months an emergency shunt between the anterior (left) hypoplastic aortic arch and the LPA after the vascular ring was divided. Total correction of the cardiovascular malformation was performed at the age of 4 years. The cardiac catheterization, angiographic and operative findings as well as the management of the case are described. Ten other patients with this combination of anomalies were found on review of the literature, eight of whom had only palliative systemic to pulmonary artery shunts. Knowledge of the morphology of the DAA by angiocardiography is helpful in planning the management. Surgical shunts when done early for the treatment of a patient with repeated anoxic spells may reduce the gravity of psychomotor retardation. A staged surgical repair of TOF is recommended for infants having hypoplastic pulmonary arteries.