Bicuspid Aortic Valve

  • Norihiko ShiiyaEmail author


Although valve repair for aortic regurgitation (AR) has gained attention and the application of individual procedures is becoming standardized according to the pathophysiology of regurgitation, repair techniques for the bicuspid aortic valve (BAV) have not yet been standardized. The main reason for this is the diversity of BAV phenotypes. Even considering just the cusps, the level of diversity in the BAV is great as one must consider the presence/absence of a raphe, the types of fused cusps, and the orientation of the commissures. In addition, BAV/AR is frequently associated with dilatation of the aortoventricular junction (AVJ), Valsalva sinus, sinotubular junction (STJ), and the ascending aorta. Thus, there are rather few cases that can be managed well simply by treating the cusps alone. In this article, taking these factors into account, the BAV phenotype, its clinical significance, various repair techniques, and problems specific to BAV are discussed.


  1. 1.
    Carpentier A. Cardiac valve surgery—the “French correction”. J Thorac Cardiovasc Surg. 1983;86:323–37.Google Scholar
  2. 2.
    Cosgrove DM, Rosenkranz ER, Hendren WG, Bartlett JC, Stewart WJ. Valvuloplasty for aortic insufficiency. J Thorac Cardiovasc Surg. 1991;102:571–6. discussion 76–7Google Scholar
  3. 3.
    Cabrol C, Cabrol A, Guiraudon G, Bertrand M. Treatment of aortic insufficiency by means of aortic annuloplasty. Arch Mal Coeur Vaiss. 1966;59:1305–12.PubMedPubMedCentralGoogle Scholar
  4. 4.
    Aicher D, Kunihara T, Abou Issa O, Brittner B, Gräber S, Schäfers H-J. Valve configuration determines long-term results after repair of the bicuspid aortic valve. Circulation. 2011;123:178–85.CrossRefGoogle Scholar
  5. 5.
    Navarra E, El Khoury G, Glineur D, Boodhwani M, Van Dyck M, Vanoverschelde JL, et al. Effect of annulus dimension and annuloplasty on bicuspid aortic valve repair. Eur J Cardiothorac Surg. 2013;44:316–22. discussion 22–3CrossRefGoogle Scholar
  6. 6.
    Vallabhajosyula P, Komlo C, Szeto WY, Wallen TJ, Desai N, Bavaria JE. Root stabilization of the repaired bicuspid aortic valve: subcommissural annuloplasty versus root reimplantation. Ann Thorac Surg. 2014;97:1227–34.CrossRefGoogle Scholar
  7. 7.
    Aicher D, Schneider U, Schmied W, Kunihara T, Tochii M, Schäfers H-J. Early results with annular support in reconstruction of the bicuspid aortic valve. J Thorac Cardiovasc Surg. 2013;145:S30–4.CrossRefGoogle Scholar
  8. 8.
    Svensson LG, Al Kindi AH, Vivacqua A, Pettersson GB, Gillinov AM, Mihaljevic T, et al. Long-term durability of bicuspid aortic valve repair. Ann Thorac Surg. 2014;97:1539–47. discussion 48CrossRefGoogle Scholar
  9. 9.
    de Kerchove L, Boodhwani M, Glineur D, Vandyck M, Vanoverschelde J-L, Noirhomme P, et al. Valve sparing-root replacement with the reimplantation technique to increase the durability of bicuspid aortic valve repair. J Thorac Cardiovasc Surg. 2011;142:1430–8.CrossRefGoogle Scholar
  10. 10.
    Schneider U, Hofmann C, Aicher D, Takahashi H, Miura Y, Schäfers HJ. Suture annuloplasty significantly improves the durability of bicuspid aortic valve repair. Ann Thorac Surg. 2017;103:504–10.CrossRefGoogle Scholar
  11. 11.
    Boodhwani M, de Kerchove L, Glineur D, Poncelet A, Rubay J, Astarci P, et al. Repair-oriented classification of aortic insufficiency: impact on surgical techniques and clinical outcomes. J Thorac Cardiovasc Surg. 2009;137:286–94.CrossRefGoogle Scholar
  12. 12.
    Fernández B, Durán AC, Fernández-Gallego T, Fernández MC, Such M, Arqué JM, et al. Bicuspid aortic valves with different spatial orientations of the leaflets are distinct etiological entities. J Am Coll Cardiol. 2009;54:2312–8.CrossRefGoogle Scholar
  13. 13.
    Sievers HH, Schmidtke C. A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg. 2007;133:1226–33.CrossRefGoogle Scholar
  14. 14.
    Sabet HY, Edwards WD, Tazelaar HD, Daly RC. Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases. Mayo Clin Proc. 1999;74:14–26.CrossRefGoogle Scholar
  15. 15.
    Jermihov P, Jia L, Sacks M, Gorman R, Gorman J III, Chandran K. Effect of geometry on the leaflet stresses in simulated models of congenital bicuspid aortic valves. Cardiovasc Eng Technol. 2011;2:48–56.CrossRefGoogle Scholar
  16. 16.
    Sadee AS, Becker AE, Verheul HA, Bouma B, Hoedemaker G. Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation. Br Heart J. 1992;67:439–41.CrossRefGoogle Scholar
  17. 17.
    Schaefer BM, Lewin MB, Stout KK, Gill E, Prueitt A, Byers PH, et al. The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape. Heart. 2008;94:1634–8.CrossRefGoogle Scholar
  18. 18.
    Fazel SS, Mallidi HR, Lee RS, Sheehan MP, Liang D, Fleischman D et al. The aortopathy of bicuspid aortic valve disease has distinctive patterns and usually involves the transverse aortic arch. J Thorac Cardiovasc Surg. 2008;135:901–7, 907.e1–2.CrossRefGoogle Scholar
  19. 19.
    Vahanian A, Alfieri O, Andreotti F, Antunes MJ, Baron-Esquivias G, Baumgartner H, et al. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J. 2012;33:2451–96.CrossRefGoogle Scholar
  20. 20.
    Eleid MF, Forde I, Edwards WD, Maleszewski JJ, Suri RM, Schaff HV, et al. Type A aortic dissection in patients with bicuspid aortic valves: clinical and pathological comparison with tricuspid aortic valves. Heart. 2013;99:1668–74.CrossRefGoogle Scholar
  21. 21.
    Park CB, Greason KL, Suri RM, Michelena HI, Schaff HV, Sundt TM 3rd. Fate of nonreplaced sinuses of Valsalva in bicuspid aortic valve disease. J Thorac Cardiovasc Surg. 2011;142:278–84.CrossRefGoogle Scholar
  22. 22.
    Sievers HH, Stierle U, Hachmann RM, Charitos EI. New insights in the association between bicuspid aortic valve phenotype, aortic configuration and valve haemodynamics. Eur J Cardiothorac Surg. 2016;49:439–46.CrossRefGoogle Scholar
  23. 23.
    Duran C, Kumar N, Gometza B, al Halees Z. Indications and limitations of aortic valve reconstruction. Ann Thorac Surg. 1991;52:447–53. discussion 53–4CrossRefGoogle Scholar
  24. 24.
    Vallabhajosyula P, Szeto WY, Komlo CM, Ryan LP, Wallen TJ, Gorman RC, et al. Geometric orientation of the aortic neoroot in patients with raphed bicuspid aortic valve disease undergoing primary cusp repair and a root reimplantation procedure. Eur J Cardiothorac Surg. 2014;45:174–80. discussion 80CrossRefGoogle Scholar
  25. 25.
    Gleason TG. Bicuspid aortic valve repair by complete conversion from “raphe’d” (type 1) to “symmetric” (type 0) morphology. J Thorac Cardiovasc Surg. 2014;148:2862–8.e1–2.CrossRefGoogle Scholar
  26. 26.
    Boodhwani M, de Kerchove L, Glineur D, El Khoury G. A simple method for the quantification and correction of aortic cusp prolapse by means of free margin plication. J Thorac Cardiovasc Surg. 2010;139:1075–7.CrossRefGoogle Scholar
  27. 27.
    Schafers HJ, Langer F, Glombitza P, Kunihara T, Fries R, Aicher D. Aortic valve reconstruction in myxomatous degeneration of aortic valves: are fenestrations a risk factor for repair failure? J Thorac Cardiovasc Surg. 2010;139:660–4.CrossRefGoogle Scholar
  28. 28.
    David TE, Feindel CM, Webb GD, Colman JM, Armstrong S, Maganti M. Long-term results of aortic valve-sparing operations for aortic root aneurysm. J Thorac Cardiovasc Surg. 2006;132:347–54.CrossRefGoogle Scholar
  29. 29.
    Ashikhmina E, Sundt TM 3rd, Dearani JA, Connolly HM, Li Z, Schaff HV. Repair of the bicuspid aortic valve: a viable alternative to replacement with a bioprosthesis. J Thorac Cardiovasc Surg. 2010;139:1395–401.CrossRefGoogle Scholar
  30. 30.
    Price J, De Kerchove L, Glineur D, Vanoverschelde JL, Noirhomme P, El Khoury G. Risk of valve-related events after aortic valve repair. Ann Thorac Surg. 2013;95:606–12. discussion 13CrossRefGoogle Scholar
  31. 31.
    Alsoufi B, Borger MA, Armstrong S, Maganti M, David TE. Results of valve preservation and repair for bicuspid aortic valve insufficiency. J Heart Valve Dis. 2005;14:752–8. discussion 58–9Google Scholar

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© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  1. 1.First Department of SurgeryHamamatsu University School of MedicineHamamatsuJapan

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