A bicuspid aortic valve (BAV) often causes aortic stenosis (AS) or regurgitation (AR). In 54 patients with a BAV (48 +/- 16 years), transthoracic and transesophageal echo were performed to measure aortic annulus diameter (AAD), to evaluate the severity of aortic valve disease (AVD) and to calculate the area eccentricity index (AEI) of a BAV defined as a ratio of the larger aortic cusp area to a smaller aortic cusp area. By multiple linear regression analysis, the severity of AR correlated significantly with the AAD (r = 0.38) and AEI (r = 0.35) (P < 0.05) and that of AS correlated significantly with the AAD (r =-0.40) and AEI (r = 0.34) (P < 0.05). Thirty-six patients showed anteroposteriorly (A-P) located BAVs and 18 patients showed right-left (R-L) located BAVs. The AAD was larger in A-P type than in R-L type (15 +/- 3 vs 13 +/- 2 mm/BSA, P < 0.05) and there was no difference in the age and AEI between the two groups. AR was more severe in A-P type than in R-L type while AS was more severe in R-L type than in A-P type (P < 0.05). Twenty-nine patients showed raphes. The AEI was larger in raphe (+) type than in raphe (-) type (1.83 +/- 0.53 vs 1.51 +/- 0.47, P < 0.05) and there was no difference in the AAD and severity of AVD between the two groups. In conclusion, a BAV with larger aortic annulus or A-P located will tend to cause AR while a BAV with smaller aortic annulus or R-L located will tend to cause AS.