The bicuspid aortic valve

  • J. F. Matthias Bechtel
  • M. Misfeld
  • C. Schmidtke
  • Hans-H. Sievers


The bicuspid aortic valve (BAV) is the most common congenital cardiac malformation. Despite being a seemingly simple and harmless anatomic variation, BAV is said to cause more morbidity than any other congenital cardiac defect [52]. BAV may lead to aortic valve stenosis (AS) or regurgitation (AR), endocarditis, an ascending aortic aneurysm, and/or devastating dissection or rupture. Although these potential consequences of BAV were first described long ago [19, 35, 36], only recently have clinicians become fully aware that the presence of a BAV poses a serious health risk. However, the so-called bicuspid aortic valve syndrome [14] is extremely heterogeneous with some patients having rapidly progressive valve and/or aortic disease, while some individuals with BAV remain free of complications throughout their lifetime. In this article, we review current concepts regarding etiology, pathomechanisms, diagnosis, and treatment of BAV with special emphasis on topics relevant for cardiac surgeons.


Aortic Valve Aortic Aneurysm Bicuspid Aortic Valve Aortic Valve Stenosis Aortic Valve Disease 
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  1. 1.
    Bauer M, Gliech V, Siniawski H, Hetzer R (2006) Configuration of the ascending aorta in patients with bicuspid and tricuspid aortic valve disease undergoing aortic valve replacement with or without reduction aortoplasty. J Heart Valve Dis 15:594–600PubMedGoogle Scholar
  2. 2.
    Bechtel JFM, Noack F, Sayk F, Erasmi AW, Bartels C, Sievers HH (2003) Histopathological grading of ascending aortic aneurysm: Comparison of patients with bicuspid versus tricuspid aortic valves. J Heart Valve Dis 12:54–61Google Scholar
  3. 3.
    Bonow RO, Carabello BA, Kanu C et al (2006) ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. Circulation 114:e84–e231CrossRefPubMedGoogle Scholar
  4. 4.
    Boodhwani M, de Kerchove L, Glineur D et al (2009) Repair-oriented classification of aortic insufficiency: impact on surgical techniques and clinical outcomes. J Thorac Cardiovasc Surg 137:286–294CrossRefPubMedGoogle Scholar
  5. 5.
    Choudhury N, Bouchot O, Rouleau L et al (2009) Local mechanical and structural properties of healthy and diseased human ascending aorta tissue. Cardiovasc Pathol 18:83–91CrossRefPubMedGoogle Scholar
  6. 6.
    Cotrufo M, Della CA, De Santo LS et al (2005) Different patterns of extracellular matrix protein expression in the convexity and the concavity of the dilated aorta with bicuspid aortic valve: preliminary results. J Thorac Cardiovasc Surg 130:504–511PubMedGoogle Scholar
  7. 7.
    Cripe L, Andelfinger G, Martin LJ, Shooner K, Benson DW (2004) Bicuspid aortic valve is heritable. J Am Coll Cardiol 44:138–143CrossRefPubMedGoogle Scholar
  8. 8.
    David TE, Omran A, Ivanov J et al (2000) Dilation of the pulmonary autograft after the Ross procedure. J Thorac Cardiovasc Surg 119:210–220CrossRefPubMedGoogle Scholar
  9. 9.
    de Sa M, Moshkovitz Y, Butany J, David TE (1999) Histologic abnormalities of the ascending aorta and pulmonary trunk in patients with bicuspid aortic valve disease: clinical relevance to the Ross procedure. J Thorac Cardiovasc Surg 118:588–596CrossRefPubMedGoogle Scholar
  10. 10.
    Della CA, Bancone C, Quarto C et al (2007) Predictors of ascending aortic dilatation with bicuspid aortic valve: a wide spectrum of disease expression. Eur J Cardiothorac Surg 31:397–404CrossRefGoogle Scholar
  11. 11.
    Della CA, Quarto C, Bancone C et al (2008) Spatiotemporal patterns of smooth muscle cell changes in ascending aortic dilatation with bicuspid and tricuspid aortic valve stenosis: focus on cell-matrix signaling. J Thorac Cardiovasc Surg 135:8–18CrossRefGoogle Scholar
  12. 12.
    Ellison JW, Yagubyan M, Majumdar R et al (2007) Evidence of genetic locus heterogeneity for familial bicuspid aortic valve. J Surg Res 142:28–31CrossRefPubMedGoogle Scholar
  13. 13.
    Erasmi AW, Sievers HH, Bechtel JF, Hanke T, Stierle U, Misfeld M (2007) Remodeling or reimplantation for valve-sparing aortic root surgery? Ann Thorac Surg 83:S752–S756CrossRefPubMedGoogle Scholar
  14. 14.
    Fedak PW (2008) Bicuspid aortic valve syndrome: heterogeneous but predictable. Eur Heart J 29:432–433CrossRefPubMedGoogle Scholar
  15. 15.
    Fedak PW, David TE, Borger M, Verma S, Butany J, Weisel RD (2005) Bicuspid aortic valve disease: recent insights in pathophysiology and treatment. Expert Rev Cardiovasc Ther 3:295–308CrossRefPubMedGoogle Scholar
  16. 16.
    Fedak PW, de Sa MP, Verma S et al (2003) Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation. J Thorac Cardiovasc Surg 126:797–806CrossRefPubMedGoogle Scholar
  17. 17.
    Friedman T, Mani A, Elefteriades JA (2008) Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert Rev Cardiovasc Ther 6:235–248CrossRefPubMedGoogle Scholar
  18. 18.
    Garg V, Muth AN, Ransom JF et al (2005) Mutations in NOTCH1 cause aortic valve disease. Nature 437:270–274CrossRefPubMedGoogle Scholar
  19. 19.
    Gore I, Seiwert VJ (1952) Dissecting aneurysm of the aorta; pathological aspects; an analysis of eighty-five cases. AMA Arch Pathol 53:121–141PubMedGoogle Scholar
  20. 20.
    Gray GW, Salisbury DA, Gulino AM (1995) Echocardiographic and color flow Doppler findings in military pilot applicants. Aviat Space Environ Med 66:32–34PubMedGoogle Scholar
  21. 21.
    Huntington K, Hunter AGW, Chan KL (1997) A prospective study to assess the frequency of familial clustering of congenital bicuspid aortic valve. J Am Coll Cardiol 30:1809–1812CrossRefPubMedGoogle Scholar
  22. 22.
    Kappetein AP, Gittenberger-de Groot AC, Zwinderman AH, Rohmer J, Poelmann RE, Huysmans HA (1991) The neural crest as a possible pathogenetic factor in coarctation of the aorta and bicuspid aortic valve. J Thorac Cardiovasc Surg 102:830–836PubMedGoogle Scholar
  23. 23.
    Kirby ML, Waldo KL (1990) Role of neural crest in congenital heart disease. Circulation 82:332–340PubMedGoogle Scholar
  24. 24.
    Le Douarin NM, Teillet MA (1974) Experimental analysis of the migration and differentiation of neuroblasts of the autonomic nervous system and of neuroectodermal mesenchymal derivates, using biologic cell marking techniques. Dev Biol 41:162–184CrossRefPubMedGoogle Scholar
  25. 25.
    Maron BJ, Hutchins GM (1974) The development of the semilunar valves in the human heart. Am J Pathol 74:331–344PubMedGoogle Scholar
  26. 26.
    Martin LJ, Ramachandran V, Cripe LH et al (2007) Evidence in favor of linkage to human chromosomal regions 18q, 5q and 13q for bicuspid aortic valve and associated cardiovascular malformations. Hum Genet 121:275–284CrossRefPubMedGoogle Scholar
  27. 27.
    Michelena HI, Desjardins VA, Avierinos JF et al (2008) Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Circulation 117:2776–2784CrossRefPubMedGoogle Scholar
  28. 28.
    Mohamed SA, Aherrahrou Z, Liptau H et al (2006) Novel missense mutations (p. T596M and p.P1797H) in NOTCH1 in patients with bicuspid aortic valve. Biochem Biophys Res Commun 345:1460–1465CrossRefPubMedGoogle Scholar
  29. 29.
    Mohamed SA, Hanke T, Schlueter C, Bullerdiek J, Sievers HH (2005) Ubiquitin fusion degradation 1-like gene dysregulation in bicuspid aortic valve. J Thorac Cardiovasc Surg 130:1531–1536CrossRefPubMedGoogle Scholar
  30. 30.
    Mohaved MR, Hepner AD, Ahmadi-Kashani M (2006) Echocardiographic prevalence of bicuspid aortic valve in the population. Heart Lung Circ 15:297–299CrossRefGoogle Scholar
  31. 31.
    Nataatmadja M, West M, West J et al (2003) Abnormal extracellular matrix protein transport associated with increased apoptosis of vascular smooth muscle cells in marfan syndrome and bicuspid aortic valve thoracic aortic aneurysm. Circulation 108 Suppl 1:II329–II334PubMedGoogle Scholar
  32. 32.
    Nishimura RA, Carabello BA, Faxon DP et al (2008) ACC/AHA 2008 guideline update on valvular heart disease: focused update on infective endocarditis: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines: endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 118:887–896CrossRefPubMedGoogle Scholar
  33. 33.
    Nistri S, Basso C, Marzari C, Mormino P, Thiene G (2005) Frequency of bicuspid aortic valve in young male conscripts by echocardiogram. Am J Cardiol 96:718–721CrossRefPubMedGoogle Scholar
  34. 34.
    Nistri S, Grande-Allen J, Noale M et al (2008) Aortic elasticity and size in bicuspid aortic valve syndrome. Eur Heart J 29:472–479CrossRefPubMedGoogle Scholar
  35. 35.
    Osler W (1866) The bicuspid condition of aortic valves. Trans Assoc Am Physicians 2:185–192Google Scholar
  36. 36.
    Peacock TB (1866) On malformations of the human heart. Churchill, LondonGoogle Scholar
  37. 37.
    Roberts WC (1970) The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 26:72–82CrossRefPubMedGoogle Scholar
  38. 38.
    Roberts WC, Ko JM (2005) Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation. Circulation 111:920–925CrossRefPubMedGoogle Scholar
  39. 39.
    Robicsek F, Thubrikar MJ, Cook JW, Fowler B (2004) The congenitally bicuspid aortic valve: how does it function? Why does it fail? Ann Thorac Surg 77:177–185CrossRefPubMedGoogle Scholar
  40. 40.
    Russo CF, Cannata A, Lanfranconi M, Vitali E, Garatti A, Bonacina E (2008) Is aortic wall degeneration related to bicuspid aortic valve anatomy in patients with valvular disease? J Thorac Cardiovasc Surg 136:937–942CrossRefPubMedGoogle Scholar
  41. 41.
    Sabet HY, Edwards WE, Tazelaar HD, Daly RC (1999) Congenitally Bicuspid Aortic Valves: A Surgical Pathology Study of 542 Cases (1991 Through 1996) and a Literature Review of 2715 Additional Cases. Mayo Clin Proc 74:14–26CrossRefPubMedGoogle Scholar
  42. 42.
    Sans-Coma V, Fernández B, Durán AC et al (1996) Fusion of valve cushions as a key factor in the formation of congenital bicuspid aortic valves in Syrian hamsters. Anat Rec 244:490–498CrossRefPubMedGoogle Scholar
  43. 43.
    Schäfers HJ, Aicher D, Langer F, Lausberg HF (2007) Preservation of the bicuspid aortic valve. Ann Thorac Surg 83:S740–S745CrossRefPubMedGoogle Scholar
  44. 44.
    Schmidtke C, Bechtel M, Hueppe M, Sievers HH (2001) Time course of aortic valve function and root dimensions after subcoronary Ross procedure for bicuspid versus tricuspid aortic valve disease. Circulation 104 (Suppl):I21–I24PubMedGoogle Scholar
  45. 45.
    Schmidtke C, Poppe D, Dahmen G, Sievers HH (2005) Echocardiographic and hemodynamic characteristics of reconstructed bicuspid aortic valves at rest and exercise. Z Kardiol 94:437–444CrossRefPubMedGoogle Scholar
  46. 46.
    Sievers HH (2004) Reflections on reduction ascending aortoplasty’s liveliness. J Thorac Cardiovasc Surg 128:499–501CrossRefPubMedGoogle Scholar
  47. 47.
    Sievers HH, Hanke T, Stierle U et al (2006) A Critical Reappraisal of the Ross Operation. Renaissance of the subcoronary implantation technique? Circulation 114[suppl I]:I-504–I-511CrossRefGoogle Scholar
  48. 48.
    Sievers HH, Schmidtke C (2007) A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg 133:1226–1233CrossRefPubMedGoogle Scholar
  49. 49.
    Stefani L, Galanti G, Toncelli L et al (2008) Bicuspid aortic valve in competitive athletes. Br J Sports Med 42:31–35CrossRefPubMedGoogle Scholar
  50. 50.
    Strader JR, Harrell TW, Adair A, Kruyer WB (2008) Efficacy of echocardiographic screening of pilot applicants. Aviat Space Environ Med 79:514–517CrossRefPubMedGoogle Scholar
  51. 51.
    Tzemos N, Therrien J, Yip J et al (2008) Outcomes in adults with bicuspid aortic valves. JAMA 300:1317–1325CrossRefPubMedGoogle Scholar
  52. 52.
    Ward C (2000) Clinical significance of the bicuspid aortic valve. Heart 83:81–85CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2010

Authors and Affiliations

  • J. F. Matthias Bechtel
    • 1
  • M. Misfeld
  • C. Schmidtke
  • Hans-H. Sievers
    • 1
  1. 1.Klinik für ChirurgieUniversitätsklinikum Schleswig-Holstein Campus LübeckLübeckGermany

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