Primary repair of interrupted aortic arch and associated heart lesions was performed in 13 patients aged from 1 to 85 days. The surgery was performed through the midline sternotomy approach in extracorporeal circulation and deep hypothermia. Hypothermic circulatory arrest at 14 to 19 degrees C was used for reconstruction of the aortic arch. In all patients it was possible to perform a direct anastomosis between the ascendent and descendent aorta. At the same time closure of the ventricular septal defect was performed in 11 patients, closure of the atrial septal defect in 4, correction of persistent truncus arteriosus in 3, resection of subaortic stenosis in 2, arterial switch repair of transposition of the great arteries in 1, correction of double outlet right ventricle in 1 and patch closure of aortico-pulmonary window in 1 patient. Three (23.1%) newborns died in the early postoperative period: two from sepsis and one from multiple organ failure. Ten patients (76.9%) were followed up for 1 to 29 months postoperatively. All of them are in very good condition with a nonrestrictive aortic anastomosis. Primary one-stage repair of interrupted aortic arch and associated heart lesions is preferred to the two-stage repair in all newborns with this critical congenital heart disease.