Bicuspid Aortic Valve

Fig. 1: Chest radiograph of a 44-year-old man shows prominence of the right cardiac contour, probably due to enlarged ascending aorta and aortic root. The overall heart size is slightly increased.
Fig. 2: Axial CT image at the level of the aortic valve shows a bicuspid valve (arrows) with calcifications of the valve leaflets (arrowheads).

Facts

• Most common congenital cardiac malformation, 1%-2% of population
• Majority of cases develop complications requiring treatment

Etiology

• Abnormal cusp formation during valvulogenesis
• May be a part of a continuum: unicuspid valve (severe form), bicuspid valve (moderate form), tricuspid valve (normal) and quadricuspid form
• May be genetic. Not clear if inheritable but studies had shown that this condition could be transmitted in autosomal dominance form, with male to female ratio of 4:1.

Complications

• Most common = aortic stenosis
• Bicuspid aortic valve very common among patients age 15-65 years with significant aortic stenosis
• The fewer the number of cusps, the greater is the chance that the valve is stenotic from birth
• Other complications: aortic insufficiency, endocarditis, aortic dilatation/aneurysm, dissection
• It is believed that changes of the aorta (dilation, aneurysm) are not secondary to valvular dysfunction, but rather a manifestation of the disease itself

Surgery if: Severe aortic insufficiency/stenosis, dilated aorta, increased left ventricular size, decreased left ventricular function

Our case: bicuspid aortic valve with aortic stenosis.

Reference:
Fedak PW, Verma S, David TE, et al. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation 2002;106:900.