We report a two-year-old boy with a rare combination of tetralogy of Fallot and aortic coarctation. The obstruction in this patient had an hourglass-like configuration, with a pressure difference of 15 mm Hg. There was a right aortic arch, bilateral brachiocephalic arteries, and persistent left superior caval vein. Percutaneous transluminal balloon angioplasty was performed to lower the resistance to the left ventricular ejection, considered a risk factor for subsequent corrective surgery of tetralogy. Six months later, he underwent combined repair of tetralogy of Fallot and coarctectomy with end-to-end anastomosis, thus avoiding the future potential for restenosis. During surgery, a ligament was found extending from the right subclavian artery to the distal side of the coarctation. The perioperative course was uneventful, indicating the success of the preoperative balloon angioplasty. We speculate that the embryogenesis of the coarctation could be related to an abnormal involution of the aortic arches.
