Table 1

Criteria for Categorizing Bicuspid Aortic Valve Disease

First Author (Ref. #)PathologyYearNumber of CasesCriteriaMain Types%
Roberts et al. (6)Surgical specimens197059Cusp orientationAnterior-posterior cusps47.5
Left-right cusps52.5
Sabet et al. (7)Pathology1999534Raphe orientationRCC and LCC Fusion52
RCC and NCC fusion7
LCC and RCC fusion2
Relative cusp size/volumeEqual cusps5
Unequal cusps92
Angelini et al. (19)Autopsy198964Leaflet spatial positionAnterior-posterior cusps64
Lateral cusps36
Number of sinuses and interleaflet triangles
Sievers et al. (17)Pathology207304Number of rapheType 0 (none)7
Type 1 (one)88
Type 2 (two)5
Spatial position of raphe/cuspAnterior-posterior cusps2
Lateral cusps4
RCC and LCC fusion71
RCC and NCC fusion15
LCC and NCC fusion3
Functional status of the valveStenosis51
Schaefer et al. (18)Echocardiogram2008192Presence and orientation of rapheType 1 (LCC and RCC fusion)79.6
Type 2 (RCC and NCC fusion)20
Type 3 (NCC and RCC fusion)0.5
Aortic root dimension and shapeType N (normal)-type 154
Type A (ascending distension)-type 239
Type E (effaced sinotubular junction)7
Michelena et al. (22)Echocardiogram2014Location of the commissuresType 1: LCC and RCC fusion70-80
Type 2: RCC and NCC fusion20-30
Type 3: LCC and NCC fusion1
Cusp symmetrySymmetric5
Kang et al. (20)MDCT2013167Cusp spatial orientation if no rapheType 2 (Anterior-Posterior)11.4
Type 5 (Right-Left)36.5
Presence and orientation of rapheType 1 (RCC and LCC fusion)44.3
Type 3 (RCC and NCC fusion)7.2
Type 4 (LCC and NCC fusion)0.6
Degree of bicuspid aortopathyType 0 (normal aorta)26.9
Type 1 (dilated aortic root)20.4
Type 2 (dilated tubular portion of ascending aorta)29.3
Type 3 (diffuse involvement including ascending aorta and arch)23.4
Jilaihawi et al. (21)MDCT201591Leaflet morphologyTricommissural23.1
Bicommissural non-raphe type20.9
Bicommissural raphe type54.9
Leaflet orientationCoronary cusp fusion
Coronary/noncoronary cusp fusion

LCC = left coronary cusp; MDCT = multidetector computed tomography; NCC = noncoronary cusp; RCC = right coronary cusp.