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Review
. 2023 Oct 25;8(1):100227.
doi: 10.1016/j.shj.2023.100227. eCollection 2024 Jan.

Bicuspid Aortic Valve Disease: Classifications, Treatments, and Emerging Transcatheter Paradigms

Ankur Kalra  1 , Rajiv Das  2 , Mohammad Alkhalil  2 , Iryna Dykun  3 , Alessandro Candreva  4 , Omar Jarral  5 , Syed M Rehman  6 , Monil Majmundar  7 , Kunal N Patel  8 , Josep Rodes-Cabau  9 , Michael J Reardon  10 , Rishi Puri  3
Affiliations
Review

Bicuspid Aortic Valve Disease: Classifications, Treatments, and Emerging Transcatheter Paradigms

Ankur Kalra et al. Struct Heart. .

Abstract

Bicuspid aortic valve (BAV) is a common congenital valvular malformation, which may lead to early aortic valve disease and bicuspid-associated aortopathy. A novel BAV classification system was recently proposed to coincide with transcatheter aortic valve replacement being increasingly considered in younger patients with symptomatic BAV, with good clinical results, yet without randomized trial evidence. Procedural technique, along with clinical outcomes, have considerably improved in BAV patients compared with tricuspid aortic stenosis patients undergoing transcatheter aortic valve replacement. The present review summarizes the novel BAV classification systems and examines contemporary surgical and transcatheter approaches.

Keywords: Aortic stenosis; Bicuspid aortic valve; Surgical aortic valve replacement; Transcatheter aortic valve replacement.

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Conflict of interest statement

Dr Josep Rodes-Cabau has reported institutional research grants from 10.13039/100006520Edwards Lifesciences, 10.13039/100004374Medtronic, and 10.13039/100000046Abbott. Dr Michael J. Reardon has received consultation fees from Medtronic, Boston Scientific, Gore Medical, and Abbott medical paid to his department. Dr Rishi Puri is a consultant to Medtronic, Boston Scientific, Philips, Shockwave Medical, Products & Features, V-Dyne, VahatiCor, Advanced NanoTherapies, NuevoSono, TherOx, Bioventrix and Centerline Biomedical. No other disclosures were reported.

Figures

Figure 1
Figure 1
Classification systems for BAV. (a) Schematic (top panel) and computed tomography images (bottom panel) of each type of Sievers classification. (b) This more novel proposed system is based on number of commissures (2 or 3), and in the presence of 2 commissures, the presence or absence of a raphe. Abbreviation: BAV, bicuspid aortic valve.
Figure 2
Figure 2
A new international consensus classification of bicuspid aortic valve. (a) represents the fused BAV type with symmetrical phenotype based on the wide angle of the nonfused non coronary cusp or asymmetrical phenotype with angulation of less than 160°. (b) represents the 2-Sinus BAV with its 2 phenotypes, anteroposterior, and lateral-lateral. (c) represents partial fusion BAV whereby 2 commissures are fused by <50%. Abbreviation: BAV, bicuspid aortic valve.
Figure 3
Figure 3
Potential mechanism of higher rate of pacemaker in BAV. (a) Aortic valve complex in a BAV Sievers 1 configuration with R-L fusion with calcium. (b) The asymmetrical TAVR expansion resulting from resistant calcific raphe and leaflet fusion may compress the non-coronary cusp toward the conduction fiber pathway along the central fibrous body. Abbreviations: BAV, bicuspid aortic valve, TAVR, transcatheter aortic valve replacement.
Figure 4
Figure 4
Transcatheter heart valve sizing based on aortic root anatomy. In bicuspid annuli that have diameters similar (tubed) or less (flared) than the intercommissural distance (ICD), valve sizing can be based simply off the annular dimensions as in tricuspid aortic valve stenosis. When the ICD is smaller than the annular diameter (tapered), valve sizing based off the ICD should be considered.
See this image and copyright information in PMC

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