Table 1:

Heterogeneous bicuspid aortic valve nomenclature

Author and yearType of studyNumber of patientsNomenclatureAdditional comments

Roberts [4] 1970

Pathology85

Anterior–posterior cusps

Right–left cusps

Presence of raphe

Discussed differentiating congenital BAV versus acquired

Brandenburg et al. [37] 1983

Echocardiography115

Clock-face nomenclature:

Commissures at 4–10 o’clock with raphe at 2 o’clock (R-L)

Commissures at 1–6 o’clock with raphe at 10 o’clock (RN)

Commissures at 3–9 o’clock without raphe (L-N)

Noted different sizes of the resulting 2 functional cusps

Angelini et al. [31] 1989

Pathology64

Anterior–posterior cusps

Right–left cusps

Presence of raphe

Noted presence of 2 (true BAV) versus 3 sinuses

Sabet et al. [32] 1999

Pathology534

RL

RN

LN

Presence of raphe

Noted symmetry of cusps: equal, unequal, thirds

Sievers and Schmidtke [34] 2007

Pathology304

Type 0 (no raphe): anteroposterior or lateral cusps (true BAV)

Type 1 (1 raphe):

R-L, RN, L-N

Type 2 (2 raphes): L-R, RN

Noted type 2 morphology associated with more aortic aneurysms

Schaefer et al. [33] 2008

Echocardiography186

Type 1: RL

Type 2: RN

Type 3: LN

Presence of raphe

Aorta:

Type N: normal shape

Type E: sinus effacement

Type A: ascending aorta dilatation

Noted type 1 BAV was associated with type N aorta with dilated root

Noted type 2 BAV associated with type A aorta

Kang et al. [30] 2013

Computed tomography167

Anteroposterior orientation:

type 1: R-L with raphe type; 2: R-L without raphe

Right–left orientation:

Type 3: RN with raphe

Type 4: L-N with raphe

Type 5: symmetrical cusps with 1 coronary artery originating from each cusp

Aorta:

Type 0: normal

Type 1: dilated root

Type 2: dilated ascending aorta

Type 3: diffuse involvement of the ascending aorta and arch

Noted AS and type 3 aorta more commonly in right–left orientation and AR and type N aorta more commonly in anteroposterior orientation

Michelena et al. [2] 2014

EchocardiographyMultiple studies

BAVCon nomenclature:

Type 1: R-L

Type 2: RN

Type 3: L-N

Presence of raphe

Noted symmetry of cusps and presence of 2 (true BAV) or 3 sinuses

Noted predominant ascending aorta dilatation in all BAV and the existence of ‘root phenotype’

Jilaihawi et al. [35] 2016

Computed tomography130

Tricommissural: functional or acquired bicuspidity of a trileaflet valve

Bicommissural with raphe

Bicommissural without raphe

Noted no association between nomenclature and TAVR complications

Sun et al. [36] 2017

Echocardiography681

Dichotomous nomenclature:

R-L

Mixed: (RN or L-N)

Noted mixed phenotype was associated with AS and surgery of the aorta

Good interobserver variability of phenotypes

Murphy et al. [38] 2017

Cardiac magnetic resonance386

Clock-face nomenclature:

Type 0: partial fusion/eccentric leaflet?

Type 1: RN, RL, LN

partial fusion/eccentric leaflet?

Type 2: RL and RN, RL and LN, RN and LN partial fusion/eccentric leaflet?

Noted partial fusion and/or eccentric leaflet
Author and yearType of studyNumber of patientsNomenclatureAdditional comments

Roberts [4] 1970

Pathology85

Anterior–posterior cusps

Right–left cusps

Presence of raphe

Discussed differentiating congenital BAV versus acquired

Brandenburg et al. [37] 1983

Echocardiography115

Clock-face nomenclature:

Commissures at 4–10 o’clock with raphe at 2 o’clock (R-L)

Commissures at 1–6 o’clock with raphe at 10 o’clock (RN)

Commissures at 3–9 o’clock without raphe (L-N)

Noted different sizes of the resulting 2 functional cusps

Angelini et al. [31] 1989

Pathology64

Anterior–posterior cusps

Right–left cusps

Presence of raphe

Noted presence of 2 (true BAV) versus 3 sinuses

Sabet et al. [32] 1999

Pathology534

RL

RN

LN

Presence of raphe

Noted symmetry of cusps: equal, unequal, thirds

Sievers and Schmidtke [34] 2007

Pathology304

Type 0 (no raphe): anteroposterior or lateral cusps (true BAV)

Type 1 (1 raphe):

R-L, RN, L-N

Type 2 (2 raphes): L-R, RN

Noted type 2 morphology associated with more aortic aneurysms

Schaefer et al. [33] 2008

Echocardiography186

Type 1: RL

Type 2: RN

Type 3: LN

Presence of raphe

Aorta:

Type N: normal shape

Type E: sinus effacement

Type A: ascending aorta dilatation

Noted type 1 BAV was associated with type N aorta with dilated root

Noted type 2 BAV associated with type A aorta

Kang et al. [30] 2013

Computed tomography167

Anteroposterior orientation:

type 1: R-L with raphe type; 2: R-L without raphe

Right–left orientation:

Type 3: RN with raphe

Type 4: L-N with raphe

Type 5: symmetrical cusps with 1 coronary artery originating from each cusp

Aorta:

Type 0: normal

Type 1: dilated root

Type 2: dilated ascending aorta

Type 3: diffuse involvement of the ascending aorta and arch

Noted AS and type 3 aorta more commonly in right–left orientation and AR and type N aorta more commonly in anteroposterior orientation

Michelena et al. [2] 2014

EchocardiographyMultiple studies

BAVCon nomenclature:

Type 1: R-L

Type 2: RN

Type 3: L-N

Presence of raphe

Noted symmetry of cusps and presence of 2 (true BAV) or 3 sinuses

Noted predominant ascending aorta dilatation in all BAV and the existence of ‘root phenotype’

Jilaihawi et al. [35] 2016

Computed tomography130

Tricommissural: functional or acquired bicuspidity of a trileaflet valve

Bicommissural with raphe

Bicommissural without raphe

Noted no association between nomenclature and TAVR complications

Sun et al. [36] 2017

Echocardiography681

Dichotomous nomenclature:

R-L

Mixed: (RN or L-N)

Noted mixed phenotype was associated with AS and surgery of the aorta

Good interobserver variability of phenotypes

Murphy et al. [38] 2017

Cardiac magnetic resonance386

Clock-face nomenclature:

Type 0: partial fusion/eccentric leaflet?

Type 1: RN, RL, LN

partial fusion/eccentric leaflet?

Type 2: RL and RN, RL and LN, RN and LN partial fusion/eccentric leaflet?

Noted partial fusion and/or eccentric leaflet

AR: aortic regurgitation; AS: aortic stenosis; BAV: bicuspid aortic valve; BAVCon: bicuspid aortic valve consortium; LN: left non-coronary fusion; RL: right–left fusion; RN: right non-coronary fusion; TAVR: transcatheter aortic valve replacement.

Table 1:

Heterogeneous bicuspid aortic valve nomenclature

Author and yearType of studyNumber of patientsNomenclatureAdditional comments

Roberts [4] 1970

Pathology85

Anterior–posterior cusps

Right–left cusps

Presence of raphe

Discussed differentiating congenital BAV versus acquired

Brandenburg et al. [37] 1983

Echocardiography115

Clock-face nomenclature:

Commissures at 4–10 o’clock with raphe at 2 o’clock (R-L)

Commissures at 1–6 o’clock with raphe at 10 o’clock (RN)

Commissures at 3–9 o’clock without raphe (L-N)

Noted different sizes of the resulting 2 functional cusps

Angelini et al. [31] 1989

Pathology64

Anterior–posterior cusps

Right–left cusps

Presence of raphe

Noted presence of 2 (true BAV) versus 3 sinuses

Sabet et al. [32] 1999

Pathology534

RL

RN

LN

Presence of raphe

Noted symmetry of cusps: equal, unequal, thirds

Sievers and Schmidtke [34] 2007

Pathology304

Type 0 (no raphe): anteroposterior or lateral cusps (true BAV)

Type 1 (1 raphe):

R-L, RN, L-N

Type 2 (2 raphes): L-R, RN

Noted type 2 morphology associated with more aortic aneurysms

Schaefer et al. [33] 2008

Echocardiography186

Type 1: RL

Type 2: RN

Type 3: LN

Presence of raphe

Aorta:

Type N: normal shape

Type E: sinus effacement

Type A: ascending aorta dilatation

Noted type 1 BAV was associated with type N aorta with dilated root

Noted type 2 BAV associated with type A aorta

Kang et al. [30] 2013

Computed tomography167

Anteroposterior orientation:

type 1: R-L with raphe type; 2: R-L without raphe

Right–left orientation:

Type 3: RN with raphe

Type 4: L-N with raphe

Type 5: symmetrical cusps with 1 coronary artery originating from each cusp

Aorta:

Type 0: normal

Type 1: dilated root

Type 2: dilated ascending aorta

Type 3: diffuse involvement of the ascending aorta and arch

Noted AS and type 3 aorta more commonly in right–left orientation and AR and type N aorta more commonly in anteroposterior orientation

Michelena et al. [2] 2014

EchocardiographyMultiple studies

BAVCon nomenclature:

Type 1: R-L

Type 2: RN

Type 3: L-N

Presence of raphe

Noted symmetry of cusps and presence of 2 (true BAV) or 3 sinuses

Noted predominant ascending aorta dilatation in all BAV and the existence of ‘root phenotype’

Jilaihawi et al. [35] 2016

Computed tomography130

Tricommissural: functional or acquired bicuspidity of a trileaflet valve

Bicommissural with raphe

Bicommissural without raphe

Noted no association between nomenclature and TAVR complications

Sun et al. [36] 2017

Echocardiography681

Dichotomous nomenclature:

R-L

Mixed: (RN or L-N)

Noted mixed phenotype was associated with AS and surgery of the aorta

Good interobserver variability of phenotypes

Murphy et al. [38] 2017

Cardiac magnetic resonance386

Clock-face nomenclature:

Type 0: partial fusion/eccentric leaflet?

Type 1: RN, RL, LN

partial fusion/eccentric leaflet?

Type 2: RL and RN, RL and LN, RN and LN partial fusion/eccentric leaflet?

Noted partial fusion and/or eccentric leaflet
Author and yearType of studyNumber of patientsNomenclatureAdditional comments

Roberts [4] 1970

Pathology85

Anterior–posterior cusps

Right–left cusps

Presence of raphe

Discussed differentiating congenital BAV versus acquired

Brandenburg et al. [37] 1983

Echocardiography115

Clock-face nomenclature:

Commissures at 4–10 o’clock with raphe at 2 o’clock (R-L)

Commissures at 1–6 o’clock with raphe at 10 o’clock (RN)

Commissures at 3–9 o’clock without raphe (L-N)

Noted different sizes of the resulting 2 functional cusps

Angelini et al. [31] 1989

Pathology64

Anterior–posterior cusps

Right–left cusps

Presence of raphe

Noted presence of 2 (true BAV) versus 3 sinuses

Sabet et al. [32] 1999

Pathology534

RL

RN

LN

Presence of raphe

Noted symmetry of cusps: equal, unequal, thirds

Sievers and Schmidtke [34] 2007

Pathology304

Type 0 (no raphe): anteroposterior or lateral cusps (true BAV)

Type 1 (1 raphe):

R-L, RN, L-N

Type 2 (2 raphes): L-R, RN

Noted type 2 morphology associated with more aortic aneurysms

Schaefer et al. [33] 2008

Echocardiography186

Type 1: RL

Type 2: RN

Type 3: LN

Presence of raphe

Aorta:

Type N: normal shape

Type E: sinus effacement

Type A: ascending aorta dilatation

Noted type 1 BAV was associated with type N aorta with dilated root

Noted type 2 BAV associated with type A aorta

Kang et al. [30] 2013

Computed tomography167

Anteroposterior orientation:

type 1: R-L with raphe type; 2: R-L without raphe

Right–left orientation:

Type 3: RN with raphe

Type 4: L-N with raphe

Type 5: symmetrical cusps with 1 coronary artery originating from each cusp

Aorta:

Type 0: normal

Type 1: dilated root

Type 2: dilated ascending aorta

Type 3: diffuse involvement of the ascending aorta and arch

Noted AS and type 3 aorta more commonly in right–left orientation and AR and type N aorta more commonly in anteroposterior orientation

Michelena et al. [2] 2014

EchocardiographyMultiple studies

BAVCon nomenclature:

Type 1: R-L

Type 2: RN

Type 3: L-N

Presence of raphe

Noted symmetry of cusps and presence of 2 (true BAV) or 3 sinuses

Noted predominant ascending aorta dilatation in all BAV and the existence of ‘root phenotype’

Jilaihawi et al. [35] 2016

Computed tomography130

Tricommissural: functional or acquired bicuspidity of a trileaflet valve

Bicommissural with raphe

Bicommissural without raphe

Noted no association between nomenclature and TAVR complications

Sun et al. [36] 2017

Echocardiography681

Dichotomous nomenclature:

R-L

Mixed: (RN or L-N)

Noted mixed phenotype was associated with AS and surgery of the aorta

Good interobserver variability of phenotypes

Murphy et al. [38] 2017

Cardiac magnetic resonance386

Clock-face nomenclature:

Type 0: partial fusion/eccentric leaflet?

Type 1: RN, RL, LN

partial fusion/eccentric leaflet?

Type 2: RL and RN, RL and LN, RN and LN partial fusion/eccentric leaflet?

Noted partial fusion and/or eccentric leaflet

AR: aortic regurgitation; AS: aortic stenosis; BAV: bicuspid aortic valve; BAVCon: bicuspid aortic valve consortium; LN: left non-coronary fusion; RL: right–left fusion; RN: right non-coronary fusion; TAVR: transcatheter aortic valve replacement.

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