The patient in this case was a boy aged 2 years and 9 months. The patient was transferred to our hospital with ductal shock, and bilateral pulmonary artery banding was performed on the 9th day after the diagnosis of interruption of the aortic arch, ventricular septal defect, subaortic stenosis, and bicuspid aortic valve. Left ventricular outflow tract stenosis due to aortic annulus diameter and subaortic stenosis after repair was suspected. Damus-Kaye-Stansel (DKS) anastomosis, extended aortic arch anastomosis, and a right modified Blalock-Taussig operation were performed. Preoperative examination of the intracardiac repair showed growth of the aortic annulus and confirmed that biventricular repair was possible after DKS take-down. The patient's native aortic and pulmonary valves were preserved, and an intracardiac repair was performed without using an extracardiac conduit. The postoperative course was uneventful, and the patient is currently in a good condition at the age of 6 years. Three and a half years after surgical intervention, echocardiography and cardiac catheterization showed improvement of subaortic stenosis and enlargement of the aortic annulus. Our findings indicate that the most appropriate surgical procedure can be selected by detailed examination of the preoperative condition at each stage of the staged operation.