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
Thirds2
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
Insufficiency39
Both9
None1
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
Nonsymmetric95
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.