ABSTRACT
Aortic valve stenosis occurs in 3-6% of patients with congenital heart disease with males being aected 3-5 times as oen as females. e anatomic types of aortic valve stenosis include unicuspid, bicuspid, tricuspid, quadricuspid, and undierentiated aortic valves. Bicuspid aortic valve is the most common malformation of the aortic valve and is present in up to 1.3% of the general population and in 70-85% of pediatric patients with aortic stenosis. Most neonates requiring intervention have either a unicuspid or a severely stenotic bicuspid valve. Aortic stenosis sometimes occurs within single families but the pattern of occurrence appears to be multifactorial. e recurrence risk in the ospring of an aected father is approximately 3% whereas it is 15% if the mother is aected. Bicuspid aortic valve and coarctation of the aorta are the most common cardiac anomalies in patients with Turner’s syndrome (monosomy XO).1
