Twenty infants with interrupted aortic arch associated with various intracardiac anomalies underwent primary complete repair using pulsatile high-flow cardiopulmonary bypass with a short period of circulatory arrest. Age at repair ranged from 11 to 126 days (mean age, 43 days). Weight ranged from 2.2 to 5.5 kg (mean weight, 3.4 kg). Associated cardiac lesions included ventricular septal defect (14 patients), truncus arteriosus (3), transposition of the great arteries (2), and aortopulmonary window (1 patient). Left ventricular outflow tract obstruction was relieved in 2 patients. The aortic arch was reconstructed with a polytetrafluoroethylene graft in 7 patients and by direct anastomosis in the most recent 11 patients. An arterial cannulation method has been devised to facilitate direct anastomosis between the ascending aorta and the descending aorta, to lessen circulatory arrest time, and to prevent dangerous laceration and postoperative narrowing of the thin, small ascending aorta at the cannulation site. Two patients died, a surgical mortality rate of 10%. There has been one late death, which was due to severe truncal valve insufficiency. The other patients are doing well with a mean follow-up of 3 years 6 months. Restenosis of the direct anastomosis has not been noted in any patient. However, subaortic stenosis with pressure gradients of 30 to 40 mm Hg developed in 3 patients. In conclusion, one-stage repair including direct anastomosis for the aortic arch reconstruction and repair of all coexisting intracardiac defects is thought to be the treatment of choice.
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