Curriculum · Cardiovascular and Hypertension
Bicuspid aortic valve
What it is
The bicuspid aortic valve is the most common congenital cardiac anomaly, 1 in 50. By age 45, half of the patients who have one will have significant aortic stenosis. The two valve lesions it causes are what to know. Calcification and fibrosis of a congenitally bicuspid aortic valve is a cause of aortic stenosis, with calcification and degeneration of a congenitally normal valve in the old patient and rheumatic valvular disease. A congenital bicuspid aortic valve is also one of the common causes of aortic regurgitation, with aortic root dilation and postinflammatory disease.
Causes and risk
Turner syndrome is the association to know. The most common cardiac defect in Turner syndrome is bicuspid aortic valve, then coarctation of the aorta, given as bicuspid aortic valve in 15% and coarctation of the aorta in 5-10%. It sits with the shield chest and widely spaced nipples in the same list of features, with short stature, webbed neck, cubitus valgus and lymphedema of hands and feet. A bicuspid aortic valve is also one of the risk factors listed for aortic dissection, and coarctation of the aorta is another entry on that same list.