ABSTRACT
In childhood, a bicuspid aortic valve (BAV) may be stenotic or insufficient; however, its function is normal in most cases, resulting in underestimation of the incidence of BAV in childhood. Aortic valve stenosis below 60 years of age occurs most often secondary to congenital BAV, which degenerates earlier than aortic valve with three cusps. Aortic stenosis in BAV may be present at birth, or may develop in childhood, adolescence or adulthood, and is usually of progressive nature. BAV is the most frequent congenital anomaly occurring in 1–2% of the population at large with predominance in men. Chronic aortic regurgitation is caused by malcoaptation of the aortic cusps due to the aortic valve involvement, or normal aortic valve with dilatation of the aortic annulus and sino-tubular junction, or a combination of both. Patients with Marfan’s syndrome, or BAV and ascending aorta dilatation, receive beta blockers to slow down progression of aortic dilatation; however, the bradycardizing effect may worsen aortic regurgitation.
