We experienced a case of ascending aortic stenosis after interrupted aortic arch repair. At the first operation in the neonatal period, diameter of the aortic annulus was 4.8 mm and no subvalvular stenosis was presented. Aortic arch was reconstructed with conventional technique of extended end-to-end anastomosis. Although ascending aortic stenosis was observed at the time, that had not been severe, however, the stenosis was exacerbated with time. At the catheterization 9 months after the operation, pressure gradient between aortic root and descending aorta was 72 mmHg. Therefore reoperation was carried out. Ascending aortoplasty using aoutologous pulmonary patch was performed. Pulmonary artery was repaired with aotologous pericardial patch. Despite mild pulmonary stenosis was observed, the postoperative course was favorable.

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