Skip to main content

Advertisement

Log in

Approach to the patient with bicuspid aortic valve and ascending aorta aneurysm

  • Published:
Current Treatment Options in Cardiovascular Medicine Aims and scope Submit manuscript

Opinion statement

Bicuspid aortic valve (BAV) disease is a common congenital heart valve abnormality accounting for a large number of valve replacements in the United States. Although still incompletely understood, the natural history of BAV disease is severe aortic stenosis and associated ascending aortic dilatation. In addition to the increased risk of endocarditis, aortic dissection and severe aortic valve dysfunction are responsible for most fatal complications. Thus, early and precise recognition of this condition is mandatory. The new American College of Cardiology/American Heart Association recommendations highlight the role of MRI and CT as complimentary tools to echocardiography for the diagnosis and surveillance of the morphology of the aortic valve and ascending aorta. Moreover, better understanding of the cellular mechanisms, including inflammation, bone formation, atherosclerotic-like processes, and aortic wall abnormalities, as well as the heritability and genetic predisposition for the disease, will define the potential for targeted medical therapies in the future. Currently, the treatment of this condition is primarily surgical. Although combined valve and ascending aorta replacement has been the most common surgical approach in the past, the increased cumulative risk of thrombotic and embolic events among these young patients has led to more conservative approaches. Several valve-sparing approaches with comparable mid-term results compared with the classic procedures have recently been reported. However, longer follow-up studies will be helpful to better define the advantages of these new surgical options. After a quick overview of the natural history of the BAV, this article provides an updated approximation of the current knowledge of the pathophysiology as well as the recommendations for the management and treatment of this disease.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

  1. Roberts WC: The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 1970, 26:72–83.

    Article  PubMed  CAS  Google Scholar 

  2. Larson EW, Edwards WD: Risk factors for aortic dissection: a necropsy study of 161 cases. Am J Cardiol 1984, 53:849–855.

    Article  PubMed  CAS  Google Scholar 

  3. Mills P, Leech G, Davies M, Leathan A: The natural history of a non-stenotic bicuspid aortic valve. Br Heart J 1978, 40:951–957.

    PubMed  CAS  Google Scholar 

  4. Lamas CC, Eykyn SJ: Bicuspid aortic valve—a silent danger: analysis of 50 cases of infective endocarditis. Clin Infect Dis 2000, 30:336–341.

    Article  PubMed  CAS  Google Scholar 

  5. Beppu S, Suzuki S, Matsuda H, et al.: Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valves. Am J Cardiol 1993, 71:322–327.

    Article  PubMed  CAS  Google Scholar 

  6. 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, 111:920–925. This article illustrates the natural history of BAV and its progression to aortic valve stenosis. It describes the prevalence of BAV in adults with aortic stenosis referred for valve replacement.

    Article  PubMed  Google Scholar 

  7. Rajamannan NM, Subramaniam M, Rickard D, et al.: Human aortic valve calcification is associated with an osteoblast phenotype. Circulation 2003, 107:2181–2184.

    Article  PubMed  Google Scholar 

  8. Rajamannan NM, Sangiorgi G, Springett M, et al.: Experimental hypercholesterolemia induces apoptosis in the aortic valve. J Heart Valve Dis 2001, 10:371–374.

    PubMed  CAS  Google Scholar 

  9. Caira FC, Stock SR, Gleason TG, et al.: Human degenerative valve disease is associated with up-regulation of low-density lipoprotein receptor-related protein 5 receptor-mediated bone formation. J Am Coll Cardiol 2006, 47:1707–1712.

    Article  PubMed  CAS  Google Scholar 

  10. Cecconi M, Manfrin M, Moraca A, et al.: Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction. Am J Cardiol 2005, 95:292–294.

    Article  PubMed  Google Scholar 

  11. Hahn RT, Roman MJ, Mogtader AH, Devereux RB: Association of aortic dilation with regurgitant, stenotic and functionally normal bicuspid aortic valves. J Am Coll Cardiol 1992, 19:283–288.

    Article  PubMed  CAS  Google Scholar 

  12. de Sa M, Moshkovitz Y, Butany J, David TE: 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, 118:588–594.

    Article  PubMed  Google Scholar 

  13. 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.

    PubMed  CAS  Google Scholar 

  14. 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, 127:686–691.

    Article  PubMed  CAS  Google Scholar 

  15. Koullias GJ, Korkolis DP, Ravichandran P, et al.: Tissue microarray detection of matrix metalloproteinases, in diseased tricuspid and bicuspid aortic valves with or without pathology of the ascending aorta. Eur J Cardiothorac Surg 2004, 26:1098–1103.

    Article  PubMed  Google Scholar 

  16. 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, 30:1809–1812.

    Article  PubMed  CAS  Google Scholar 

  17. Lee TC, Zhao YD, Courtman DW, Stewart DJ: Abnormal aortic valve development in mice lacking endothelial nitric oxide synthase. Circulation 2000, 101:2345–2348.

    PubMed  CAS  Google Scholar 

  18. Garg V, Muth AN, Ransom JF, et al.: Mutations in NOTCH1 cause aortic valve disease. Nature 2005, 437:270–274.

    Article  PubMed  CAS  Google Scholar 

  19. Brandenburg RO Jr, Tajik AJ, Edwards WD, et al.: Accuracy of 2-dimensional echocardiographic diagnosis of congenitally bicuspid aortic valve: echocardiographicanatomic correlation in 115 patients. Am J Cardiol 1983, 51:1469–1473.

    Article  PubMed  Google Scholar 

  20. Quenot JP, Boichot C, Petit A, et al.: Usefulness of MRI in the follow-up of patients with repaired aortic coarctation and bicuspid aortic valve. Int J Cardiol 2005, 103:312–316.

    Article  PubMed  Google Scholar 

  21. John AS, Dill T, Brandt RR, et al.: Magnetic resonance to assess the aortic valve area in aortic stenosis: how does it compare to current diagnostic standards? J Am Coll Cardiol 2003, 42:519–526.

    Article  PubMed  Google Scholar 

  22. Caruthers SD, Lin SJ, Brown P, et al.: Practical value of cardiac magnetic resonance imaging for clinical quantification of aortic valve stenosis: comparison with echocardiography. Circulation 2003, 108:2236–2243.

    Article  PubMed  Google Scholar 

  23. Rosenhek R, Rader F, Loho N, et al.: Statins but not angiotensin-converting enzyme inhibitors delay progression of aortic stenosis. Circulation 2004, 110:1291–1295.

    Article  PubMed  CAS  Google Scholar 

  24. Novaro GM, Tiong IY, Pearce GL, et al.: Effect of hydroxymethylglutaryl coenzyme a reductase inhibitors on the progression of calcific aortic stenosis. Circulation 2001, 104:2205–2209.

    PubMed  CAS  Google Scholar 

  25. Shores J, Berger KR, Murphy EA, Pyeritz RE: Progression of aortic dilatation and the benefit of long-term betaadrenergic blockade in Marfan’s syndrome. N Engl J Med 1994, 330:1335–1341.

    Article  PubMed  CAS  Google Scholar 

  26. Bonow RO, Carabello B, Chatteerjee K, 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 Develop Guidelines for the Management of Patients With Valvular Heart Disease). J Am Coll Cardiol 2006, 48:e1-e148. This is the most recent recommendation from the ACC and AHA for the management and surgical treatment of patients with BAV and ascending aortic dilatation.

    Article  PubMed  Google Scholar 

  27. Dellgren G, David TE, Raanani E, et al.: Late hemodynamic and clinical outcomes of aortic valve replacement with the Carpentier-Edwards Perimount pericardial bioprosthesis. J Thorac Cardiovasc Surg 2002, 124:146–154.

    Article  PubMed  Google Scholar 

  28. Dellgren G, David TE, Raanani E, et al.: The Toronto SPV: hemodynamic data at 1 and 5 years’ postimplantation. Semin Thorac Cardiovasc Surg 1999, 11(4 suppl 1):107–113.

    PubMed  CAS  Google Scholar 

  29. Gaudiani VA, Grunkemeier GL, Castro LJ, et al.: The risks and benefits of reoperative aortic valve replacement. Heart Surg Forum 2004, 7:E170-E173.

    Article  PubMed  Google Scholar 

  30. Cosgrove DM, Rosenkranz ER, Hendren WG, et al.: Valvuloplasty for aortic insufficiency. J Thorac Cardiovasc Surg 1991, 102:571–576; discussion 576–577.

    PubMed  CAS  Google Scholar 

  31. Casselman FP, Gillinov AM, Akhrass R, et al.: Intermediate-term durability of bicuspid aortic valve repair for prolapsing leaflet. Eur J Cardiothorac Surg 1999, 15:302–308.

    Article  PubMed  CAS  Google Scholar 

  32. Rao V, Van Arsdell GS, David TE, et al.: Aortic valve repair for adult congenital heart disease: a 22-year experience. Circulation 2000, 102(19 suppl 3):III40-III43.

    PubMed  CAS  Google Scholar 

  33. Davierwala PM, David TE, Armstrong S, Ivanov J: Aortic valve repair versus replacement in bicuspid aortic valve disease. J Heart Valve Dis 2003, 12:679–686; discussion 686.

    PubMed  Google Scholar 

  34. Kouchoukos NT, Masetti P, Nickerson NJ, et al.: The Ross procedure: long-term clinical and echocardiographic follow-up. Ann Thorac Surg 2004, 78:773–781; discussion 781.

    Article  PubMed  Google Scholar 

  35. Luciani GB, Casali G, Favaro A, et al.: Fate of the aortic root late after Ross operation. Circulation 2003, 108(suppl 1):II61-II67.

    PubMed  Google Scholar 

  36. Elefteriades JA: Natural history of thoracic aortic aneurysms: indications for surgery, and surgical versus nonsurgical risks. Ann Thorac Surg 2002, 74:S1877-S1880; discussion S1892–S1898.

    Article  PubMed  Google Scholar 

  37. Boyer JK, Gutierrez F, Braverman AC: Approach to the dilated aortic root. Curr Opin Cardiol 2004, 19:563–569.

    Article  PubMed  Google Scholar 

  38. Sundt TM 3rd, Mora BN, Moon MR, et al.: Options for repair of a bicuspid aortic valve and ascending aortic aneurysm. Ann Thorac Surg 2000, 69:1333–1337.

    Article  PubMed  Google Scholar 

  39. Borger MA, Preston M, Ivanov J, et al.: Should the ascending aorta be replaced more frequently in patients with bicuspid aortic valve disease? J Thorac Cardiovasc Surg 2004, 128:677–683.

    Article  PubMed  Google Scholar 

  40. Kuralay E, Demirkilic U, Arslan M, Tatar H: Surgical approach to ascending aorta in patients with bicuspid aortic valve. Ann Thorac Surg 2004, 78:757.

    Article  PubMed  Google Scholar 

  41. Bentall H, De Bono A: A technique for complete replacement of the ascending aorta. Thorax 1968, 23:338–339.

    Article  PubMed  CAS  Google Scholar 

  42. Hagl C, Strauch JT, Spielvogel D, et al.: Is the Bentall procedure for ascending aorta or aortic valve replacement the best approach for long-term event-free survival? Ann Thorac Surg 2003, 76:698–703; discussion 703.

    Article  PubMed  Google Scholar 

  43. Aicher D, Langer F, Kissinger A, et al.: Valve-sparing aortic root replacement in bicuspid aortic valves: a reasonable option? J Thorac Cardiovasc Surg 2004, 128:662–668.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ortiz, J.T., Shin, D.D. & Rajamannan, N.M. Approach to the patient with bicuspid aortic valve and ascending aorta aneurysm. Curr Treat Options Cardio Med 8, 461–467 (2006). https://doi.org/10.1007/s11936-006-0034-6

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11936-006-0034-6

Keywords

Navigation