The incidence of bicuspid aortic valve is common, however, only small percentage of such individuals develop aortic stenosis during childhood years. Bicuspid aortic valve may have associated ascending aortic dilation, regardless of the level of valve disease. Aortic stenosis may occur in association with other left heart lesions, such as mitral stenosis and coarctation of the aorta. Aortic stenosis is typically asymptomatic unless the stenosis is severe or critical causing compromise of cardiac output. Ejection systolic murmur in aortic stenosis is preceded by a click and is best heard over the right upper sternal border, radiating into the suprasternal region and neck. Second heart sound may be narrowly split due to delay in aortic valve closure or even paradoxically split (splitting in expiration and single in inspiration).