Congenital supra-aortic stenoses are (1) annular narrowing of the root of the ascending aorta (2) supra-aortic diaphragmatic stenosis (3) long segmental ascending aortic dysplasia. Its main lesion is a restrictive short segmental luminal stenosis or occlusion of the aorta resulting in impaired aortic blood flow. The majority (more than 95%) of lesions in the constricted segment of the aorta are located in the distal part of the aortic arch at the junction with the thoracic descending aorta, i.e., the aortic isthmus, adjacent to the ductus arteriosus or the arterial ligamentous area. However, in rare cases, the narrowing segment may be located in the aortic arch, the thoracic descending aorta or even the abdominal aorta. Sometimes the aorta may be narrowed in two places. Very few patients have a family history of the disease. How to effectively prevent circumferential narrowing of the root of the ascending aorta? The cause of this disease is not clear, so there are no effective preventive measures. Since this disease is often combined with aortic valve malformation, coronary artery dilatation and abnormal opening position, right ventricular outflow tract stenosis and mitral valve lesions, a detailed preoperative examination should be performed in such patients to facilitate timely management. When removing abnormal fibrous crests or septa in the aortic wall, it is important to avoid damaging the valves and coronary arteries to prevent residual cardiac malformations and serious complications after surgery. After surgery, the circulatory status should be kept stable, blood pressure should be controlled, and bleeding from the patch site should be reduced. Stable cardiac function should be maintained and arrhythmias should be prevented in order to achieve good surgical results. After the establishment of extracorporeal circulation and myocardial protection measures for limited supravalvular stenosis, the ascending aorta is blocked, and a longitudinal incision is made at the root of the ascending aorta from above the stenosis to the non-coronary sinus, and the lesion is carefully examined. If the supravalvular septum is adherent to the aortic valve, it should be carefully separated, and then the septum should be removed or the thickened aortic wall lining and fibrous tissue should be peeled off, and the coronary artery obstruction should be removed. Or the ascending aorta can be enlarged by “herringbone” incision, resection of the thickened intima and fibrous tissue, and repair of the ascending aorta with woven sutures. For extensive supravalvular stenosis, a blood catheter is inserted through the femoral artery, and the innominate artery, left common carotid artery, left subclavian artery, and aortic arch are freed. Extracorporeal circulation was established and myocardial protection measures were taken. The aorta is blocked proximal to the opening of the left subclavian artery and the left common carotid artery and the innominate artery are clamped. A long longitudinal incision is made in the ascending aorta with the lower end of the incision reaching the aortic sinus. The thickened fibrous tissue over the aortic sinus is peeled off and excised, and the thickened intima and fibrous tissue of the aortic wall is removed from the ascending aorta and the opening of the aortic arch branches, and then the ascending aortic incision is enlarged with polyester sutures. Cases of limited supravalvular aortic stenosis have low operative mortality, good long-term outcome, and disappearance of systolic pressure gradient after surgery. Extensive supravalvular stenosis cases have a slightly higher postoperative mortality if the obstructing lesion is not completely removed.