Congenital aortic regurgitationICD-11 Sub-sub category

Congenital cardiovascular malformation of the aortic valve allowing backward flow into the ventricle. Congenital aortic regurgitation/insufficiency is rare as an isolated entity. There are rare reports of congenital malformation of the aortic valve that result in aortic insufficiency shortly after birth from an absent or underdeveloped aortic valve cusp. Aortic insufficiency is more commonly seen with other associated cardiovascular anomalies: (1) in stenotic aortic valves (commonly stenotic congenital bicuspid aortic valves) with some degree of aortic regurgitation due to aortic leaflet abnormality; (2) in association with a ventricular septal defect (especially in subarterial type and is commonly seen in Asian populations); (3) secondary to aortic-left ventricular tunnel; (4) secondary to tethering or retraction of aortic valve leaflets in cases of supravalvar aortic stenosis that may involve the aortic valve; and similarly (5) secondary to encroachment on an aortic cusp by a subaortic membrane fibromuscular ridge; or (6) turbulence caused by a stenotic jet can create progressive aortic regurgitation.