Abstract
A bicuspid aortic valve (BAV) refers to a condition in which the valve has two cusps rather than three. Usually ignored, this manifests itself later in life. This bicuspid valve may manifest earlier in children with significant aortic stenosis because they have a severe left ventricular outflow tract restriction that worsens over time. This syndrome commonly results in congestive heart failure in newborns and early neonatal life. There can be a small amount of significant risk in pediatric patients having this BAV causing stenosis and being one of the reasons for sudden, unexpected death. Morphological differences result from a congenital cardiac abnormality called BAV. This paper emphasizes the importance of a multidisciplinary team in managing BAV and critical aortic stenosis and provides evaluation and treatment guidelines for both conditions. Transcatheter or surgical intervention is used for symptomatic individuals or those with a moderate to severe obstruction of the left ventricular outflow tract.