General Considerations and Etiologies of Aortic Stenosis

  • Frances O. Wood
  • Amr E. Abbas


The earliest descriptions of aortic stenosis are credited to Riverius in 1646 where he provided a clear-cut description of the observed pathological findings of calcified aortic valve cusps in association with weak and diminished peripheral pulses. Aortic stenosis was described again by Bonet in 1679, however, John Baptist Morgagni, professor of anatomy in the University of Padua, referred to aortic stenosis in 1761 and is credited in providing a brilliant description of an autopsy specimen of calcified aortic valve cusps found in a patient and suggested the valve was both stenotic and incompetent. In his description, he quoted a similar case described by Georgius Greiselius and he clarified the anatomical and pathophysiological features of acquired aortic stenosis. In 1806, Corvisart provided another impressive correlation of clinical and autopsy findings and in 1854, William Stokes provided yet another vivid description of the disease. This chapter will provide a general overview of aortic stenosis as well as a review of the common etiologies of aortic stenosis.


Aortic stenosis Etiology of aortic stenosis Left ventricular outflow tract (LVOT) Valvular aortic stenosis Supra-valvular aortic stenosis Sub-valvular aortic stenosis Epidemiology of aortic stenosis Causes of aortic stenosis 


  1. 1.
    Willius FA, Dry TJ. A history of the heart and circulation. Philadelphia: W. B. Saunders Company; 1948. p. 456.Google Scholar
  2. 2.
    Mulcahy R. The early descriptions of aortic incompetence. Br Heart J. 1961;24:633–6.CrossRefGoogle Scholar
  3. 3.
    Bailey C, Likoff W. Surgical management of aortic stenosis: an evolution of techniques and results. Arch Intern Med. 1957;99(6):859–87.CrossRefGoogle Scholar
  4. 4.
    Carabelo BA, Stewat WJ, Crawford FA. Aortic valve disease. In: Topol EJ, editor. Text book of cardiovascular medicine. 2nd ed. Philadelphia: Lippincott-Raven; 2002. p. 509–28.Google Scholar
  5. 5.
    Piazza N, de Jaegere P, Becker AE, Serruys PW, Anderson RH. Anatomy of the aortic valvular complex and its implications for transcatheter implantation of the aortic valve. Circ Cardiovasc Interv. 2008;1:74–81.PubMedCrossRefGoogle Scholar
  6. 6.
    Lung B, Baron G, Butchart EG, Delahaye F, Gohlke-Barwolf C, Levang OW, Tornos P, Vanoverschelde JL, Vermeer F, Bpersma E, Ravaud P, Vahanian A. A prospective survey of patients with valvular heart disease in Europe: the Euro heart survey on valvular heart disease. Eur Heart J. 2003;24(13):1231–43.CrossRefGoogle Scholar
  7. 7.
    Nkomo VT, Gardin JM, Skelton TN, Gottdiener JS, Scott CG, Enriquez-Sarano M. Burden of valvular heart diseases: a population based study. Lancet. 2006;368:1005–11.PubMedCrossRefGoogle Scholar
  8. 8.
    Osnabrugge RL, Mylotte D, Head SJ, Van Mieghem NM, Nkomo VT, LeReun CM, Bogers AJ, Piazza N, Kappetein AP. Aortic stenosis in the elderly disease prevalence and number of candidates for transcatheter aortic valve replacement: a meta-analysis and modeling study. J Am Coll Cardiol. 2013;62:1002–12.PubMedCrossRefGoogle Scholar
  9. 9.
    Bach DS, Siao D, Girard SE, Duvernoy C, McCallister Jr BD, Gualano SK. Evaluation of patients with severe symptomatic aortic stenosis who do not undergo aortic valve replacement: the potential role of subjectively overestimated operative risk. Circ Cardiovasc Qual Outcomes. 2009;2:533–9.PubMedCrossRefGoogle Scholar
  10. 10.
    Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald’s heart disease: a textbook of cardiovascular medicine. 8th ed. Philadelphia: Saunders/Elsevier; 2008.Google Scholar
  11. 11.
    Ward C. Clinical significance of the bicuspid aortic valve. Heart. 2000;83:81–5.PubMedCentralPubMedCrossRefGoogle Scholar
  12. 12.
    Rossebø AB M.D., Pedersen TR M.D., Ph.D., Boman K M.D., Ph.D., Brudi P M.D., Chambers JB M.D., Egstrup K M.D., Ph.D., Gerdts E M.D., Ph.D., Gohlke-Bärwolf C M.D., Holme I Ph.D., Kesäniemi AY M.D., Ph.D., Malbecq W Ph.D., Nienaber CA M.D., Ph.D., Ray S M.D., Skjærpe T M.D., Ph.D., Wachtell K M.D., Ph.D., Willenheimer R M.D., Ph.D. Intensive lipid lowering with simvastatin and ezetimibe in aortic stenosis. N Engl J Med. 2008;359:1343–56.PubMedCrossRefGoogle Scholar
  13. 13.
    Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol. 1970;26:72–83.PubMedCrossRefGoogle Scholar
  14. 14.
    Steinberger J, Moller JH, Berry JM, Sinaiko AR. Echocardiographic diagnosis of heart disease in apparently healthy adolescents. Pediatrics. 2000;105:815–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Emanuel R, Withers R, O’Brien K, Ross P, Feizi O. Congenitally bicuspid aortic valves. Clinicogenetic study of 41 families. Br Heart J. 1978;40:1402–7.PubMedCentralPubMedCrossRefGoogle Scholar
  16. 16.
    Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT, O’Rourke RA, Otto CM, Sha PM, Shanewise JS. 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. 2006;114:e84–231.PubMedCrossRefGoogle Scholar
  17. 17.
    Mack G, Silberbach M. Aortic and pulmonary stenosis. Pediatr Rev. 2000;21:79–85.PubMedCrossRefGoogle Scholar
  18. 18.
    Duran AC, Frescura C, Sans-Coma V, Angelini A, Basso C, Thiene G. Bicuspid aortic valves in hearts with other congenital heart disease. J Heart Valve Dis. 1995;4:581–90.PubMedGoogle Scholar
  19. 19.
    Roose-Hesselink JW, Scholzel BE, Heijdra RJ, Spitaels SE, Meijboom FJ, Boersma E, Bogers AJ, Simoons ML. Aortic valve and aortic arch pathology after coarctation repair. Heart. 2003;89:1074–7.CrossRefGoogle Scholar
  20. 20.
    Sybert VP. Cardiovascular malformations and complications in Turner syndrome. Pediatrics. 1998;101(1):E11.PubMedCrossRefGoogle Scholar
  21. 21.
    Fernandes SM, Sanders SP, Khairy P, Jenkins KJ, Gauvreau K, Lang P, Simonds H, Colan SD. Morphology of bicuspid aortic valve in children and adolescents. J Am Coll Cardiol. 2004;44:1648–51.PubMedCrossRefGoogle Scholar
  22. 22.
    Fedak PW, Verma S, David TE, Leask RL, Weisel RD, Butany J. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation. 2002;106:900–4.PubMedCrossRefGoogle Scholar
  23. 23.
    Cripe L, Andelfinger G, Martin LJ, Shooner K, Benson DW. Bicuspid aortic valve is heritable. J Am Coll Cardiol. 2004;44:138–43.PubMedCrossRefGoogle Scholar
  24. 24.
    Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules JW, Graham Jr TP, Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GD. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: 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). J Am Coll Cardiol. 2008;52:e143–263.PubMedCrossRefGoogle Scholar
  25. 25.
    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.PubMedCrossRefGoogle Scholar
  26. 26.
    Park SJ, Enriquez-Sarano M, Choi JO, Lee SC, Park SW, Kim DK, Jeon ES, Oh JK, Chang SA. Hemodynamic patterns for symptomatic presentations of severe aortic stenosis. JACC: Card Imaging. 2013;6:137–46.Google Scholar
  27. 27.
    Monin JL, Lancellotti P, Monchi M, Lim P, Weiss E, Pierard L, Gueret P. Circulation. 2009;120:69–75.PubMedCrossRefGoogle Scholar
  28. 28.
    Lim P, Monin JL, Monchi M, Garot J, Pasquet A, Hittinger L, Vanoverschelde JL, Carayon A, Gueret P. Predictors of outcome in patients with severe aortic stenosis and normal left ventricular function: role of B-type natriuretic peptide. Eur Heart J. 2004;25:2048–53.PubMedCrossRefGoogle Scholar
  29. 29.
    Bergler-Klein J, Klaar U, Heger M, Rosenhek R, Mundigler G, Gabriel H, Binder T, Pacher R, Maurer G, Baumgartner H. Natriuretic peptides predict symptom-free survival and postoperative outcome in severe aortic stenosis. Circulation. 2004;109:2302–8.PubMedCrossRefGoogle Scholar
  30. 30.
    Legget ME, Kuusisto J, Healy NL, Fujioka M, Schwaegler RG, Otto CM. Gender differences in left ventricular function at rest and with exercise in asymptomatic aortic stenosis. Am Heart J. 1996;131:94–100.PubMedCrossRefGoogle Scholar
  31. 31.
    Hachicha Z, Dumesnil JG, Bogaty P, Pibarot P. Paradoxical low-flow, low-gradient severe aortic stenosis despite preserved ejection fraction is associated with higher afterload and reduced survival. Circulation. 2007;115:2856–64.PubMedCrossRefGoogle Scholar
  32. 32.
    Cioffi G, Faggiano P, Vizzardi E, Tarantini L, Cramariuc D, Gerdts E, de Simone G. Prognostic value of inappropriately high left ventricular mass in asymptomatic severe aortic stenosis. Heart. 2011;97:301–7.PubMedCrossRefGoogle Scholar
  33. 33.
    Duncan AI, Lowe BS, Garcia MJ, Xu M, Gillinov AM, Mihaljevic T, Koch CG. Influence of concentric left ventricular remodeling on early mortality after aortic valve replacement. Ann Thorac Surg. 2008;85:2030–9.PubMedCrossRefGoogle Scholar
  34. 34.
    Orsinelli DA, Aurigemma GP, Battista S, Krendel S, Gaasch WH. Left ventricular hypertrophy and mortality after aortic valve replacement for aortic stenosis. J Am Coll Cardiol. 1993;22:1679–83.PubMedCrossRefGoogle Scholar
  35. 35.
    Fuster RG, Argudo JA, Albarova OG, Sos FH, Lopez SC, Sorli MJ, Codoner MB, Minano JA. Left ventricular mass index in aortic valve surgery: a new index for early valve replacement? Eur J Cardiothorac Surg. 2003;23:696–702.PubMedCrossRefGoogle Scholar
  36. 36.
    Mehta RH, Bruckman D, Das S, Tsai T, Russman P, Karavite D, Monaghan H, Sonnad S, Shea MJ, Eagle KA, Deeb GM. Implications of increased left ventricular mass index on in-hospital outcomes in patients undergoing aortic valve surgery. J Thorac Cardiovasc Surg. 2001;122:919–28.PubMedCrossRefGoogle Scholar
  37. 37.
    Bech-Hanssen O, Wallentin I, Houltz E, Beckman Surrkula M, Larsson S, Caidahl K. Gender differences in patients with severe aortic stenosis: impact on preoperative left ventricular geometry and function, as well as early postoperative morbidity and mortality. Eur J Cardiothorac Surg. 1999;15:24–30.PubMedCrossRefGoogle Scholar
  38. 38.
    Otto CM, Lind BK, Kitzman DW, Gersh BJ, Siscovick DS. Association of aortic-valve sclerosis with cardiovascular mortality and morbidity in the elderly. N Engl J Med. 1999;34:142–7.CrossRefGoogle Scholar
  39. 39.
    Olsen MH, Wachtell K, Bella JN, Gerdts E, Palmieri V, Nieminen MS, Smith G, Ibsen H, Devereux RB, LIFE Substudy. Aortic valve sclerosis relates to cardiovascular events in patients with hypertension (a LIFE substudy). Am J Cardiol. 2005;95:132–6.PubMedCrossRefGoogle Scholar
  40. 40.
    Taylor HA, Clark BL, Garrison RJ, Andrew ME, Han H, Fox ER, Arnett DK, Samdarshi T, Jones DW. Relation of aortic valve sclerosis to risk of coronary heart disease in African. Am J Cardiol. 2005;95(3):401–4.PubMedCrossRefGoogle Scholar
  41. 41.
    Ross J, Braunwald E. Aortic stenosis. Circulation. 1968;38:V-61–7.CrossRefGoogle Scholar
  42. 42.
    Otto CM, Burwash IG, Legget ME, Munt BI, Fujioka M, Healy NL, Kraft CD, Miyake-Hull CY, Schwaegler RG. Prospective study of asymptomatic valvular aortic stenosis clinical, echocardiographic and exercise predictors of outcome. Circulation. 1997;95:2262–70.PubMedCrossRefGoogle Scholar
  43. 43.
    Rosenhek R, Binder T, Porenta G, Lang I, Christ G, Schemper M, Maurer G, Baumgartner H. Predictors of outcome in severe, asymptomatic aortic stenosis. N Engl J Med. 2000;343:611–7.PubMedCrossRefGoogle Scholar
  44. 44.
    Pellikka PA, Sarano ME, Nishimura RA, Malouf JF, Bailey KR, Scott CG, Barnes ME, Tajik AJ. Outcome of 622 adults with asymptomatic, hemodynamically significant aortic stenosis during prolonged follow-up. Circulation. 2005;111:3290–5.PubMedCrossRefGoogle Scholar
  45. 45.
    Rosenhek R, Zilberszac R, Schemper M, Czerny M, Mundigler G, Graf S, Bergler-Klein J, Grimm M, Gabriel H, Maurer G, Makkar RR, Fontana GP, Jilaihawi H, Kapadia S, Pichard AD, Douglas PS, Thourani VH, Babaliaros VC, Webb JG, Herrmann HC, Bavaria JE, Kodali S, Brown DL, Bowers B, Dewey TM, Svensson LG, Tuzcu M, Moses JW, Williams MR, Siegel RJ, Akin JJ, Anderson WN, Pocock S, Smith CR, Leon MB, PARTNER Trial Investigators. Transcatheter aortic-valve replacement for inoperable severe aortic stenosis. N Engl J Med. 2012;366:1696–704.CrossRefGoogle Scholar
  46. 46.
    The Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC), The European Association for Cardio-Thoracic Surgery (EACTS). Guidelines on the management of valvular heart disease. Eur Heart J. 2012;33:2451–96.CrossRefGoogle Scholar
  47. 47.
    Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA, O’Gara PT, Ruiz CE, Skubas NJ, Sorajja P, Sundt TM, Thomas JD. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;129(23):2440–92.PubMedCrossRefGoogle Scholar
  48. 48.
    Ussia GP, Mule M, Barbanti M, Cammalleri V, Scarabelli M, Imme S, Capodanno D, Ciriminna S, Tamburino C. Quality of life assessment after percutaneous aortic valve implantation. Eur Heart J. 2009;30:1790–6.PubMedCrossRefGoogle Scholar
  49. 49.
    Bouma BJ, van den Brink RBA, van der Meulen JHP, Verheul HA, Cheriex EC, Hamer HPM, Dekker E, Lie KI, Tijssen JGP. To operate or not on elderly patients with aortic stenosis: the decision and its consequences. Heart. 1999;82:143–8.PubMedCentralPubMedCrossRefGoogle Scholar
  50. 50.
    Iung B, Baron G, Butchart EG, Delahaye F, Gohlke-Ba¨rwolf C, Levang OW, Tornos P, Vanoverschelde JL, Vermeer F, Boersma E, Ravaud P, Vahanian A. A prospective survey of patients with valvular heart disease in Europe: the Euro Heart Survey on valvular heart disease. Eur Heart J. 2003;24:1231–43.PubMedCrossRefGoogle Scholar
  51. 51.
    Varadarajan P, Kapoor N, Bansal RC, Pai RG. Clinical profile and natural; history of 453 nonsurgically managed patients with severe aortic stenosis. Ann Thorac Surg. 2006;82:2111–5.PubMedCrossRefGoogle Scholar
  52. 52.
    Iung B, Cachier A, Baron G, Messika-Zeitoun D, Delahaye F, Tornos P, Gohlke-Bärwolf C, Boersma E, Ravaud P, Vahanian A. Decision-making in elderly patients with severe aortic stenosis: why are so many denied surgery? Eur Heart J. 2005;26:2714–20.PubMedCrossRefGoogle Scholar
  53. 53.
    Brown JM, O’Brien SM, Wu C, Sikora JAH, Griffith BP, Gammie JS. Isolated aortic valve replacement in North America comprising 108,687 patients in 10 years: changes in risks, valve types, and outcomes in the Society of Thoracic Surgeons National Database. J Thorac Cardiovasc Surg. 2009;137:82–90.PubMedCrossRefGoogle Scholar
  54. 54.
    Kodali SK, Williams MR, Smith CR, Svensson LG, Webb JG, Makkar RR, Fontana GP, Dewey TM, Thourani VH, Pichard AD, Fischbein M, Szeto WY, Lim S, Greason KL, Teirstein PS, Malaisrie SC, Doublas PS, Hahn RT, Whisenant B, Zajarias A, Wang D, Akin JJ, Anderson WN, Leon MB, PARNTER Trial Investigators. Two-year outcomes after transcatheter or surgical aortic-valve replacement. NEJM. 2012;366:1686–95.PubMedCrossRefGoogle Scholar
  55. 55.
    Makker RR, Fontana GP, Jilaihawi H, Kapadia S, Pichard AD, Doublas PS, Thouruani VH, Babaliasros VC, Webb JC, Herrmann HC, Bavaria JE, Kodali S, Brown DL, Bowers B, Dewey TM, Svenssson LG, Tuzcu M, Moses JW, Williams MR, Siegel RJ, Akin JJ, Anderson WN, Pocock S, Smith CR, Leon MB, PARTNER Trial Investigators. Transcatheter aortic-valve replacement for inoperable severe aortic stenosis. NEJM. 2012;366:1697–704.Google Scholar
  56. 56.
    Adams DH, Popma JJ, Reardon MJ, Yakubov SJ, Coselli JS, Deeb GM, Gleason TG, Buchbinder M, Hermiller Jr J, Kleiman NS, Chetcuti S, Heiser J, Merhi W, Zorn G, Tadros P, Robinson N, Petrossian G, Hughes GC, Harrison JK, Conte J, Maini B, Mumtaz M, Chenoweth S, Oh JK, U. S. CoreValve Clinical Investigators. Transcatheter aortic-valve replacement with a self-expanding prosthesis. NEJM. 2014;370:1790–8.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London 2015

Authors and Affiliations

  1. 1.Department of Cardiovascular MedicineBeaumont Health, Oakland University/William Beaumont School of MedicineRoyal OakUSA

Personalised recommendations