Skip to main content
SpringerLink
Log in

Congenital Anomalies of the Aortic Valve

  • Chapter
  • First Online:
Aortic Regurgitation

  • 824 Accesses

Abstract

The aortic valve may exist in congenitally anomalous variants that differ in the number of valve leaflets. Most important is the bicuspid aortic valve which is relatively frequent congenital disorder. Unicuspid aortic valve is less frequent and quadricuspid aortic valve is rare. All congenitally anomalous aortic valves display aberrant haemodynamic flow pattern and, despite a considerably long asymptomatic interval, are prone to develop into a significant aortic valve disease, either a stenosis or regurgitation (or both). Interrelation of aberrant aortic valve types and aortic dilation aortopathies is well documented though yet not fully understood. A thorough knowledge of congenital aortic anomalies is essential both for proper surgical strategy regarding the valve disease and decision for preventive surgery on a dilated ascending aorta.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 99.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 139.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Basso C, Boschello M, Perrone C, et al. An echocardiographic survey of primary school children for bicuspid aortic valve. Am J Cardiol. 2004;93:661–3.

    Article  PubMed  Google Scholar 

  2. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39:1890–900.

    Article  PubMed  Google Scholar 

  3. Roger VL, Go AS, Lloyd-Jones DM, et al. Heart disease and stroke statistics—2011 update: a report from the American Heart Association. Circulation. 2011;123:e18–e209.

    Article  PubMed  Google Scholar 

  4. Tutar E, Ekici F, Atalay S, et al. The prevalence of bicuspid aortic valve in newborns by echocardiographic screening. Am Heart J. 2005;150:513–5.

    Article  PubMed  Google Scholar 

  5. Ward C. Clinical significance of the bicuspid aortic valve. Heart. 2000;83:81–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Huntington K, Hunter AG, Chan KL. A prospective study to assess the frequency of familial clustering of congenital bicuspid aortic valve. J Am Coll Cardiol. 1997;30:1809–12.

    Article  CAS  PubMed  Google Scholar 

  7. Clementi M, Notari L, Borghi A, et al. Familial congenital bicuspid aortic valve: a disorder of uncertain inheritance. Am J Med Genet. 1996;62:336–8.

    Article  CAS  PubMed  Google Scholar 

  8. Cripe L, Andelfinger G, Martin LJ, et al. Bicuspid aortic valve is heritable. J Am Coll Cardiol. 2004;44:138–43.

    Article  PubMed  Google Scholar 

  9. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). Circulation. 2008;118:2395–451.

    Article  PubMed  Google Scholar 

  10. Michelena HI, Khanna AD, Mahoney D, et al. Incidence of aortic complications in patients with bicuspid aortic valves. JAMA. 2011;306:1104–12.

    Article  CAS  PubMed  Google Scholar 

  11. Roos-Hesselink JW, Scholzel BE, Heijdra RJ, et al. Aortic valve and aortic arch pathology after coarctation repair. Heart. 2003;89:1074–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Kirby ML, Waldo KL. Role of neural crest in congenital heart disease. Circulation. 1990;82:332–40.

    Article  CAS  PubMed  Google Scholar 

  13. Kappetein AP, Gittenberger-de Groot AC, Zwinderman AH, et al. The neural crest as a possible pathogenetic factor in coarctation of the aorta and bicuspid aortic valve. J Thorac Cardiovasc Surg. 1991;102:830–6.

    CAS  PubMed  Google Scholar 

  14. Dulac Y, Pienkowski C, Abadir S, et al. Cardiovascular abnormalities in Turner’s syndrome: what prevention? Arch Cardiovasc Dis. 2008;101:485–90.

    Article  PubMed  Google Scholar 

  15. Hutchins GM, Nazarian IH, Bulkley BH. Association of left dominant coronary arterial system with congenital bicuspid aortic valve. Am J Cardiol. 1978;42:57–9.

    Article  CAS  PubMed  Google Scholar 

  16. Rosenquist TH, Beall AC, Modis L, et al. Impaired elastic matrix development in the great arteries after ablation of the cardiac neural crest. Anat Rec. 1990;226:347–59.

    Article  CAS  PubMed  Google Scholar 

  17. de Sa M, Moshkovitz Y, Butany J, et al. 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. 1999;118:588–94.

    Article  PubMed  Google Scholar 

  18. Niwa K, Perloff JK, Bhuta SM, et al. Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analyses. Circulation. 2001;103:393–400.

    Article  CAS  PubMed  Google Scholar 

  19. Fedak PW, de Sa MP, Verma S, et al. Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation. J Thorac Cardiovasc Surg. 2003;126:797–806.

    Article  PubMed  Google Scholar 

  20. Okoye MI, Mueller WF Jr, Congdon DE, et al. Isolated congenital hypoplastic bicuspid pulmonary valve. Report of a case associated with seizure disorder in a 27-year-old man. Am J Forensic Med Pathol. 1983;4:79–83.

    Article  CAS  PubMed  Google Scholar 

  21. Vistarini N, Gebhard C, Desjardins G, et al. Successful repair of a bicuspid pulmonary autograft valve causing early insufficiency after a Ross procedure. Ann Thorac Surg. 2016;101:e99–e101.

    Article  PubMed  Google Scholar 

  22. Schafers HJ, Schmied W, Marom G, et al. Cusp height in aortic valves. J Thorac Cardiovasc Surg. 2013;146:269–74.

    Article  PubMed  Google Scholar 

  23. Sievers HH, Schmidtke C. A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg. 2007;133:1226–33.

    Article  PubMed  Google Scholar 

  24. Buchner S, Hulsmann M, Poschenrieder F, et al. Variable phenotypes of bicuspid aortic valve disease: classification by cardiovascular magnetic resonance. Heart. 2010;96:1233–40.

    Article  PubMed  Google Scholar 

  25. Jackson V, Petrini J, Caidahl K, et al. Bicuspid aortic valve leaflet morphology in relation to aortic root morphology: a study of 300 patients undergoing open-heart surgery. Eur J Cardiothorac Surg. 2011;40:e118–24.

    PubMed  Google Scholar 

  26. Sabet HY, Edwards WD, Tazelaar HD, et al. 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.

    Article  CAS  PubMed  Google Scholar 

  27. Sievers HH, Stierle U, Mohamed SA, et al. Toward individualized management of the ascending aorta in bicuspid aortic valve surgery: the role of valve phenotype in 1362 patients. J Thorac Cardiovasc Surg. 2014;148:2072–80.

    Article  PubMed  Google Scholar 

  28. Michelena HI, Desjardins VA, Avierinos JF, et al. Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Circulation. 2008;117:2776–84.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Tzemos N, Therrien J, Yip J, et al. Outcomes in adults with bicuspid aortic valves. JAMA. 2008;300:1317–25.

    Article  CAS  PubMed  Google Scholar 

  30. American College of Cardiology, American Heart Association Task Force on Practice Guidelines, Society of Cardiovascular Anesthesiologists, et al. 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. J Am Coll Cardiol. 2006;48:e1–148.

    Article  Google Scholar 

  31. Wallby L, Steffensen T, Broqvist M. Role of inflammation in nonrheumatic, regurgitant heart valve disease. A comparative, descriptive study regarding apolipoproteins and inflammatory cells in nonrheumatic heart valve disease. Cardiovasc Pathol. 2007;16:171–8.

    Article  PubMed  CAS  Google Scholar 

  32. Wallby L, Janerot-Sjoberg B, Steffensen T, et al. T lymphocyte infiltration in non-rheumatic aortic stenosis: a comparative descriptive study between tricuspid and bicuspid aortic valves. Heart. 2002;88:348–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Robicsek F, Thubrikar MJ, Cook JW, et al. The congenitally bicuspid aortic valve: how does it function? Why does it fail? Ann Thorac Surg. 2004;77:177–85.

    Article  PubMed  Google Scholar 

  34. Endo M, Nabuchi A, Okuyama H, et al. Differing relationship between hypercholesterolemia and a bicuspid aortic valve according to the presence of aortic valve stenosis or aortic valve regurgitation. Gen Thorac Cardiovasc Surg. 2015;63:502–6.

    Article  PubMed  Google Scholar 

  35. Verma S, Szmitko PE, Fedak PW, et al. Can statin therapy alter the natural history of bicuspid aortic valves? Am J Physiol Heart Circ Physiol. 2005;288:H2547–9.

    Article  CAS  PubMed  Google Scholar 

  36. Chan KL. Is aortic stenosis a preventable disease? J Am Coll Cardiol. 2003;42:593–9.

    Article  CAS  PubMed  Google Scholar 

  37. Rossebo AB, Pedersen TR, Boman K, et al. Intensive lipid lowering with simvastatin and ezetimibe in aortic stenosis. N Engl J Med. 2008;359:1343–56.

    Article  PubMed  Google Scholar 

  38. McKinsey DS, Ratts TE, Bisno AL. Underlying cardiac lesions in adults with infective endocarditis. The changing spectrum. Am J Med. 1987;82:681–8.

    Article  CAS  PubMed  Google Scholar 

  39. Kahveci G, Bayrak F, Pala S, et al. Impact of bicuspid aortic valve on complications and death in infective endocarditis of native aortic valves. Tex Heart Inst J. 2009;36:111–6.

    PubMed  PubMed Central  Google Scholar 

  40. Lamas CC, Eykyn SJ. Bicuspid aortic valve—a silent danger: analysis of 50 cases of infective endocarditis. Clin Infect Dis. 2000;30:336–41.

    Article  CAS  PubMed  Google Scholar 

  41. Michelena HI, Katan O, Suri RM, et al. Incidence of infective endocarditis in patients with bicuspid aortic valves in the community. Mayo Clin Proc. 2016;91:122–3.

    Article  PubMed  Google Scholar 

  42. Gersony WM, Hayes CJ, Driscoll DJ, et al. Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation. 1993;87:I121–6.

    CAS  PubMed  Google Scholar 

  43. Nishimura RA, Carabello BA, Faxon DP, et al. 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. J Am Coll Cardiol. 2008;52:676–85.

    Article  PubMed  Google Scholar 

  44. Habib G, Lancellotti P, Antunes MJ, et al. 2015 ESC Guidelines for the management of infective endocarditis: The Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by: European Association for Cardio-Thoracic Surgery (EACTS), the European Association of Nuclear Medicine (EANM). Eur Heart J. 2015;36:3075–128.

    Article  PubMed  Google Scholar 

  45. Van der Meer JT, Van Wijk W, Thompson J, et al. Efficacy of antibiotic prophylaxis for prevention of native-valve endocarditis. Lancet. 1992;339:135–9.

    Article  PubMed  Google Scholar 

  46. Strom BL, Abrutyn E, Berlin JA, et al. Dental and cardiac risk factors for infective endocarditis. A population-based, case-control study. Ann Intern Med. 1998;129:761–9.

    Article  CAS  PubMed  Google Scholar 

  47. Della Corte A, Bancone C, Quarto C, et al. Predictors of ascending aortic dilatation with bicuspid aortic valve: a wide spectrum of disease expression. Eur J Cardiothorac Surg. 2007;31:397–404; discussion 404–5.

    Article  PubMed  Google Scholar 

  48. Beroukhim RS, Kruzick TL, Taylor AL, et al. Progression of aortic dilation in children with a functionally normal bicuspid aortic valve. Am J Cardiol. 2006;98:828–30.

    Article  PubMed  Google Scholar 

  49. Gurvitz M, Chang RK, Drant S, et al. Frequency of aortic root dilation in children with a bicuspid aortic valve. Am J Cardiol. 2004;94:1337–40.

    Article  PubMed  Google Scholar 

  50. Lewis MG. Idiopathic medionecrosis causing aortic incompetence. Br Med J. 1965;1:1478.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Erdheim J. Medionecrosis idiopathica cystica. Virch Arch Path Anat. 1929;273:454–79.

    Article  Google Scholar 

  52. Bauer M, Pasic M, Meyer R, et al. Morphometric analysis of aortic media in patients with bicuspid and tricuspid aortic valve. Ann Thorac Surg. 2002;74:58–62.

    Article  PubMed  Google Scholar 

  53. Nataatmadja M, West M, West J, et al. 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. 2003;108(Suppl 1):II329–34.

    PubMed  Google Scholar 

  54. Bonderman D, Gharehbaghi-Schnell E, Wollenek G, et al. Mechanisms underlying aortic dilatation in congenital aortic valve malformation. Circulation. 1999;99:2138–43.

    Article  CAS  PubMed  Google Scholar 

  55. Della Corte A, Quarto C, Bancone C, et al. 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. 2008;135:8–18, 18.e1-2.

    Article  CAS  PubMed  Google Scholar 

  56. Cotrufo M, Della Corte A, De Santo LS, et al. 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. 2005;130:504–11.

    Article  CAS  PubMed  Google Scholar 

  57. Schmid FX, Bielenberg K, Schneider A, et al. Ascending aortic aneurysm associated with bicuspid and tricuspid aortic valve: involvement and clinical relevance of smooth muscle cell apoptosis and expression of cell death-initiating proteins. Eur J Cardiothorac Surg. 2003;23:537–43.

    Article  PubMed  Google Scholar 

  58. Boyum J, Fellinger EK, Schmoker JD, et al. Matrix metalloproteinase activity in thoracic aortic aneurysms associated with bicuspid and tricuspid aortic valves. J Thorac Cardiovasc Surg. 2004;127:686–91.

    Article  CAS  PubMed  Google Scholar 

  59. Ikonomidis JS, Jones JA, Barbour JR, et al. Expression of matrix metalloproteinases and endogenous inhibitors within ascending aortic aneurysms of patients with bicuspid or tricuspid aortic valves. J Thorac Cardiovasc Surg. 2007;133:1028–36.

    Article  CAS  PubMed  Google Scholar 

  60. Dollery CM, McEwan JR, Henney AM. Matrix metalloproteinases and cardiovascular disease. Circ Res. 1995;77:863–8.

    Article  CAS  PubMed  Google Scholar 

  61. Hope MD, Hope TA, Meadows AK, et al. Bicuspid aortic valve: four-dimensional MR evaluation of ascending aortic systolic flow patterns. Radiology. 2010;255:53–61.

    Article  PubMed  Google Scholar 

  62. Hope MD, Sigovan M, Wrenn SJ, et al. MRI hemodynamic markers of progressive bicuspid aortic valve-related aortic disease. J Magn Reson Imaging. 2014;40:140–5.

    Article  PubMed  Google Scholar 

  63. Della Corte A, Bancone C, Conti CA, et al. Restricted cusp motion in right-left type of bicuspid aortic valves: a new risk marker for aortopathy. J Thorac Cardiovasc Surg. 2012;144:360–9, 369.e1.

    Article  PubMed  Google Scholar 

  64. Barker AJ, Lanning C, Shandas R. Quantification of hemodynamic wall shear stress in patients with bicuspid aortic valve using phase-contrast MRI. Ann Biomed Eng. 2010;38:788–800.

    Article  PubMed  Google Scholar 

  65. Barker AJ, Markl M, Burk J, et al. Bicuspid aortic valve is associated with altered wall shear stress in the ascending aorta. Circ Cardiovasc Imaging. 2012;5:457–66.

    Article  PubMed  Google Scholar 

  66. den Reijer PM, Sallee D 3rd, van der Velden P, et al. Hemodynamic predictors of aortic dilatation in bicuspid aortic valve by velocity-encoded cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2010;12:4.

    Article  Google Scholar 

  67. Della Corte A, Bancone C, Dialetto G, et al. Towards an individualized approach to bicuspid aortopathy: different valve types have unique determinants of aortic dilatation. Eur J Cardiothorac Surg. 2014;45:e118–24; discussion e124.

    Article  PubMed  Google Scholar 

  68. Della Corte A, De Santo LS, Montagnani S, et al. Spatial patterns of matrix protein expression in dilated ascending aorta with aortic regurgitation: congenital bicuspid valve versus Marfan’s syndrome. J Heart Valve Dis. 2006;15:20–7; discussion 27.

    PubMed  Google Scholar 

  69. Charitos EI, Stierle U, Petersen M, et al. The fate of the bicuspid valve aortopathy after aortic valve replacement. Eur J Cardiothorac Surg. 2014;45:e128–35.

    Article  PubMed  Google Scholar 

  70. Girdauskas E, Rouman M, Disha K, et al. The fate of mild-to-moderate proximal aortic dilatation after isolated aortic valve replacement for bicuspid aortic valve stenosis: a magnetic resonance imaging follow-up study†. Eur J Cardiothorac Surg. 2016;49:e80–7.

    Article  PubMed  Google Scholar 

  71. Biner S, Rafique AM, Ray I, et al. Aortopathy is prevalent in relatives of bicuspid aortic valve patients. J Am Coll Cardiol. 2009;53:2288–95.

    Article  PubMed  PubMed Central  Google Scholar 

  72. Abbott ME. Coarctation of the aorta. Am Heart J. 1928;3:574.

    Article  Google Scholar 

  73. Pape LA, Tsai TT, Isselbacher EM, et al. Aortic diameter >or = 5.5 cm is not a good predictor of type A aortic dissection: observations from the International Registry of Acute Aortic Dissection (IRAD). Circulation. 2007;116:1120–7.

    Article  PubMed  Google Scholar 

  74. Erbel R, Eggebrecht H. Aortic dimensions and the risk of dissection. Heart. 2006;92:137–42.

    Article  PubMed  PubMed Central  Google Scholar 

  75. Meszaros I, Morocz J, Szlavi J, et al. Epidemiology and clinicopathology of aortic dissection. Chest. 2000;117:1271–8.

    Article  CAS  PubMed  Google Scholar 

  76. Erbel R, Aboyans V, Boileau C, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J. 2014;35:2873–926.

    Article  PubMed  Google Scholar 

  77. Cosgrove DM, Rosenkranz ER, Hendren WG, et al. Valvuloplasty for aortic insufficiency. J Thorac Cardiovasc Surg. 1991;102:571–6; discussion 576–7.

    CAS  PubMed  Google Scholar 

  78. Arabkhani B, Mookhoek A, Di Centa I, et al. Reported outcome after valve-sparing aortic root replacement for aortic root aneurysm: a systematic review and meta-analysis. Ann Thorac Surg. 2015;100:1126–31.

    Article  PubMed  Google Scholar 

  79. Vohra HA, Whistance RN, De Kerchove L, et al. Valve-preserving surgery on the bicuspid aortic valve. Eur J Cardiothorac Surg. 2013;43:888–98.

    Article  PubMed  Google Scholar 

  80. Price J, De Kerchove L, Glineur D, et al. Risk of valve-related events after aortic valve repair. Ann Thorac Surg. 2013;95:606–12; discussion 613.

    Article  PubMed  Google Scholar 

  81. Aicher D, Holz A, Feldner S, et al. Quality of life after aortic valve surgery: replacement versus reconstruction. J Thorac Cardiovasc Surg. 2011;142:e19–24.

    Article  PubMed  Google Scholar 

  82. Zacek P, Holubec T, Vobornik M, et al. Quality of life after aortic valve repair is similar to Ross patients and superior to mechanical valve replacement: a cross-sectional study. BMC Cardiovasc Disord. 2016;16:63.

    Article  PubMed  PubMed Central  Google Scholar 

  83. Edwards JE. Pathologic aspects of cardiac valvular insufficiencies. AMA Arch Surg. 1958;77:634–49.

    Article  CAS  PubMed  Google Scholar 

  84. Novaro GM, Mishra M, Griffin BP. Incidence and echocardiographic features of congenital unicuspid aortic valve in an adult population. J Heart Valve Dis. 2003;12:674–8.

    PubMed  Google Scholar 

  85. Mookadam F, Thota VR, Garcia-Lopez AM, et al. Unicuspid aortic valve in adults: a systematic review. J Heart Valve Dis. 2010;19:79–85.

    PubMed  Google Scholar 

  86. Mookadam F, Thota VR, Lopez AM, et al. Unicuspid aortic valve in children: a systematic review spanning four decades. J Heart Valve Dis. 2010;19:678–83.

    PubMed  Google Scholar 

  87. Bansal A, Arora S, Traub D, et al. Unicuspid aortic valve and aortic arch aneurysm in a patient with Turner syndrome. Asian Cardiovasc Thorac Ann. 2008;16:266–7.

    Article  PubMed  Google Scholar 

  88. Miyabara S, Nakayama M, Suzumori K, et al. Developmental analysis of cardiovascular system of 45,X fetuses with cystic hygroma. Am J Med Genet. 1997;68:135–41.

    Article  CAS  PubMed  Google Scholar 

  89. Roberts WC, Ko JM. 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. 2005;111:920–5.

    Article  PubMed  Google Scholar 

  90. Agnihotri AK, Desai SC, Lai YQ, et al. Two distinct clinical presentations in adult unicuspid aortic valve. J Thorac Cardiovasc Surg. 2006;131:1169–70.

    Article  PubMed  Google Scholar 

  91. Sniecinski RM, Shanewise JS, Glas KE. Transesophageal echocardiography of a unicuspid aortic valve. Anesth Analg. 2009;108:788–9.

    Article  PubMed  Google Scholar 

  92. Roberts CS, Roberts WC. Dissection of the aorta associated with congenital malformation of the aortic valve. J Am Coll Cardiol. 1991;17:712–6.

    Article  CAS  PubMed  Google Scholar 

  93. Larson EW, Edwards WD. Risk factors for aortic dissection: a necropsy study of 161 cases. Am J Cardiol. 1984;53:849–55.

    Article  CAS  PubMed  Google Scholar 

  94. Pretre R, Kadner A, Dave H, et al. Tricuspidisation of the aortic valve with creation of a crown-like annulus is able to restore a normal valve function in bicuspid aortic valves. Eur J Cardiothorac Surg. 2006;29:1001–6.

    Article  PubMed  Google Scholar 

  95. McMullan DM, Oppido G, Davies B, et al. Surgical strategy for the bicuspid aortic valve: tricuspidization with cusp extension versus pulmonary autograft. J Thorac Cardiovasc Surg. 2007;134:90–8.

    Article  PubMed  Google Scholar 

  96. Noly PE, Basmadjian L, Bouhout I, et al. New insights into unicuspid aortic valve disease in adults: not just a subtype of bicuspid aortic valves. Can J Cardiol. 2016;32:110–6.

    Article  PubMed  Google Scholar 

  97. Schafers HJ, Aicher D, Riodionycheva S, et al. Bicuspidization of the unicuspid aortic valve: a new reconstructive approach. Ann Thorac Surg. 2008;85:2012–8.

    Article  PubMed  Google Scholar 

  98. Franciulli M, Aicher D, Radle-Hurst T, et al. Root remodeling and aortic valve repair for unicuspid aortic valve. Ann Thorac Surg. 2014;98:823–9.

    Article  PubMed  Google Scholar 

  99. Vahanian A, Alfieri O, Andreotti F, et al. Guidelines on the management of valvular heart disease (version 2012): the Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS). Eur J Cardiothorac Surg. 2012;42:S1–44.

    Article  PubMed  Google Scholar 

  100. Robicsek F, Sanger PW, Daugherty HK, et al. Congenital quadricuspid aortic valve with displacement of the left coronary orifrice. Am J Cardiol. 1969;23:288–90.

    Article  CAS  PubMed  Google Scholar 

  101. Feldman BJ, Khandheria BK, Warnes CA, et al. Incidence, description and functional assessment of isolated quadricuspid aortic valves. Am J Cardiol. 1990;65:937–8.

    Article  CAS  PubMed  Google Scholar 

  102. Loukas M, Aly I, Tubbs RS, et al. The naming game: a discrepancy among the medical community. Clin Anat. 2016;29:285–9.

    Article  PubMed  Google Scholar 

  103. Davia JE, Fenoglio JJ, DeCastro CM, et al. Quadricuspid semilunar valves. Chest. 1977;72:186–9.

    Article  CAS  PubMed  Google Scholar 

  104. Tutarel O. The quadricuspid aortic valve: a comprehensive review. J Heart Valve Dis. 2004;13:534–7.

    PubMed  Google Scholar 

  105. Lanzillo G, Breccia PA, Intonti F. Congenital quadricuspid aortic valve with displacement of the right coronary orifice. Scand J Thorac Cardiovasc Surg. 1981;15:149–51.

    Article  CAS  PubMed  Google Scholar 

  106. Kim HS, McBride RA, Titus JL. Quadricuspid aortic valve and single coronary ostium. Arch Pathol Lab Med. 1988;112:842–4.

    CAS  PubMed  Google Scholar 

  107. Possati F, Calafiore AM, Di Giammarco G, et al. Quadricuspid aortic valve and pulmonary valve stenosis. A rare combination in the adult. Minerva Cardioangiol. 1984;32:815–8.

    CAS  PubMed  Google Scholar 

  108. Iglesias A, Oliver J, Munoz JE, et al. Quadricuspid aortic valve associated with fibromuscular subaortic stenosis and aortic regurgitation treated by conservative surgery. Chest. 1981;80:327–8.

    Article  CAS  PubMed  Google Scholar 

  109. Rosenkranz ER, Murphy DJ Jr, Cosgrove DM 3rd. Surgical management of left coronary artery ostial atresia and supravalvar aortic stenosis. Ann Thorac Surg. 1992;54:779–81.

    Article  CAS  PubMed  Google Scholar 

  110. Janssens U, Klues HG, Hanrath P. Congenital quadricuspid aortic valve anomaly associated with hypertrophic non-obstructive cardiomyopathy: a case report and review of the literature. Heart. 1997;78:83–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  111. Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol. 1973;31:623–6.

    Article  CAS  PubMed  Google Scholar 

  112. Schmidt KI, Jeserich M, Aicher D, et al. Tricuspidization of the quadricuspid aortic valve. Ann Thorac Surg. 2008;85:1087–9.

    Article  PubMed  Google Scholar 

  113. Aicher D, Schafers HJ. Tricuspidization of the quadricuspid aortic valve. Multimed Man Cardiothorac Surg. 2010;2010:mmcts 2009 004051.

    Google Scholar 

  114. Song MG, Yang HS, Lee DH, et al. Mid-term results in patients having tricuspidization of the quadricuspid aortic valve. J Cardiothorac Surg. 2014;9:29.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Cardiac Surgery, Charles University, Faculty of Medicine in Hradec Králové, University Hospital Hradec Králové, Hradec Králové, Czech Republic

    Jan Vojacek M.D., Ph.D., Pavel Zacek M.D., Ph.D. & Jan Dominik M.D., Ph.D.

  2. Department of Cardiac Surgery, Kerckhoff Heart and Lung Centre, Bad Nauheim, Germany

    Tomas Holubec M.D., Ph.D.

Authors
  1. Jan Vojacek M.D., Ph.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Pavel Zacek M.D., Ph.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jan Dominik M.D., Ph.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Tomas Holubec M.D., Ph.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding authors

Correspondence to Jan Vojacek M.D., Ph.D. or Pavel Zacek M.D., Ph.D. .

Editor information

Editors and Affiliations

  1. Department of Cardiac Surgery, Charles University in Prague, Hradec Kralove, Czech Republic

    Jan Vojacek

  2. Department of Cardiac Surgery, Charles University in Prague, Hradec Kralove, Czech Republic

    Pavel Zacek

  3. Department of Cardiac Surgery, Charles University in Prague, Hradec Kralove, Czech Republic

    Jan Dominik

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Vojacek, J., Zacek, P., Dominik, J., Holubec, T. (2018). Congenital Anomalies of the Aortic Valve. In: Vojacek, J., Zacek, P., Dominik, J. (eds) Aortic Regurgitation. Springer, Cham. https://doi.org/10.1007/978-3-319-74213-7_6

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-74213-7_6

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-74212-0

  • Online ISBN: 978-3-319-74213-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation