Most cases of congenital aortic stenosis present with symptoms of aortic outlet stenosis only in childhood. Angina pectoris is more common because of the early onset of coronary atherosclerotic lesions. Some patients have a family history. Signs are similar to those of other types of aortic outlet stenosis, but no systolic karate sounds are heard. Heart murmurs and tremors are higher than in valvular stenosis, and aortic diastolic murmurs are rare. Some patients have poor growth, short stature, low intelligence, verbosity, and special facial features: receding jaw, anterior nostrils, low nasal bridge, thick lips, wide forehead, large eye spacing, and poor dental occlusion. About 5% of patients have increased blood calcium. x-ray and electrocardiogram show signs similar to those of other types of aortic outlet stenosis. Cross-sectional echocardiography: It can directly show the site and length of supravalvular stenosis. Cases of supra-aortic stenosis, especially congenital aortic stenosis with mental retardation, peculiar facial features, and extensive pulmonary stenosis, often result in sudden death in early life due to severe obstruction of the left ventricular outflow tract and coronary artery disease. Cases that are not treated surgically rarely grow into adulthood. Cardiac catheterization: Left heart catheterization with serial recording of the pressure curve may reveal a pressure waveform change over the aorta. Selective left ventriculography may show the site, length, and severity of supravalvular stenosis, while also looking for normal morphology and function of the aortic valve, as well as the coronary sinus and coronary artery. A right heart angiogram can show whether the common pulmonary artery and its branches are also diseased. Because of the risk of cardiac insufficiency and other risks after catheterization and angiography in many patients, aortic-enhanced CT is now recommended for preoperative testing. It can simultaneously detect possible pulmonary stenosis, which is also a risk factor for surgery.