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Biscuspid Aortic Valve Repair

  • Nhue Do
  • Joel PriceEmail author
  • Lars G. Svensson
Chapter

Abstract

Bicuspid aortic valve (BAV) disease is the most common congenital defect with a prevalence of 1–2% in the population. Patients with BAV are at risk to develop a number of conditions including aortic complications, aortic valve stenosis and aortic valve insufficiency (AI). Patients with severe, symptomatic aortic insufficiency experienced increased risk of heart failure and death. While aortic valve replacement is a highly effective treatment that remains the criterion standard, aortic valve repair (AVR) offers a number of potential advantages. The development of effective and durable techniques, for leaflet repair and aortic annulus reconstruction, has enabled the repair of the regurgitant aortic valve in selected patients. The primary goal of AVR is to restore a durable surface of coaptation to the regurgitant valve. A detailed understanding of the functional anatomy of the aortic root and mechanisms of AI are critical. A number of techniques have been developed to reconstruct these valves including annuloplasty and leaflet repairs. A number of experienced centers have reported durable long-term results, with good long-term survival and freedom from reoperation beyond 15 years in at some centers. Long term comparison between repair and biological valve replacements have shown that beyond approximately 11 years, patients with repairs have a lower risk of both reoperation and mortality.

Keywords

Bicuspid aortic valve Aortic valve repair Aortic regurgitation Coaptation Sinotubular junction Aortoventricular junction Leaflet repair Prolapse Annuloplasty Aortic valve-sparing root replacement David procedure 

References

  1. 1.
    Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol. 1970;26:72–83.CrossRefGoogle Scholar
  2. 2.
    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
  3. 3.
    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.CrossRefGoogle Scholar
  4. 4.
    Fernandes SM, Khairy P, Sanders SP, et al. Bicuspid aortic valve morphology and interventions in the young. J Am Coll Cardiol. 2007;49:2211–4.CrossRefGoogle Scholar
  5. 5.
    Robledo-Carmona J, Rodríguez-Bailón I, Carrasco-Chinchilla F, Fernández B, Jiménez-Navarro M, Porras-Martin C, et al. Hereditary patterns of bicuspid aortic valve in a hundred families. Int J Cardiol. 2013;168:3443–9.CrossRefGoogle Scholar
  6. 6.
    Cripe L, Andelfinger G, Martin LJ, Shooner K, Benson DW. Bicuspid aortic valve is heritable. J Am Coll Cardiol. 2004;44:138–43.CrossRefGoogle Scholar
  7. 7.
    Larson EW, Edwards WD. Risk factors for aortic dissection: a necropsy study of 161 cases. Am J Cardiol. 1984;53:849–55.CrossRefGoogle Scholar
  8. 8.
    Olson LJ, Subramanian R, Edwards WD. Surgical pathology of pure aortic insufficiency: a study of 225 cases. Mayo Clin Proc. 1984;59:835–41.CrossRefGoogle Scholar
  9. 9.
    Detaint D, Michelena HI, Nkomo VT, Vahanian A, Jondeau G, Sarano ME. Aortic dilatation patterns and rates in adults with bicuspid aortic valves: a comparative study with Marfan syndrome and degenerative aortopathy. Heart. 2014;100:126–34.CrossRefGoogle Scholar
  10. 10.
    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.CrossRefGoogle Scholar
  11. 11.
    Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Guyton RA, et al. American College of Cardiology/American Heart Association Task Force on Practice Guidelines. 2014 AHA/ACC guideline 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. J Am Coll Cardiol. 2014;63:e57–185.CrossRefGoogle Scholar
  12. 12.
    McKellar SH, Michelena HI, Li Z, Schaff HV, Sundt TM 3rd. Long-term risk of aortic events following aortic valve replacement in patients with bicuspid aortic valves. Am J Cardiol. 2010;106:1626–33.CrossRefGoogle Scholar
  13. 13.
    Borger MA, David TE. Management of the valve and ascending aorta in adults with bicuspid aortic valve disease. Semin Thorac Cardiovasc Surg. 2005;17:143–7.CrossRefGoogle Scholar
  14. 14.
    Goland S, Czer LS, De Robertis MA, Mirocha J, Kass RM, Fontana GP, Chang W, Trento A. Risk factors associated with reoperation and mortality in 252 patients after aortic valve replacement for congenitally bicuspid aortic valve disease. Ann Thorac Surg. 2007;83:931–7.CrossRefGoogle Scholar
  15. 15.
    Svensson LG, Blackstone EH, Cosgrove DM 3rd. Surgical options in young adults with aortic valve disease. Curr Probl Cardiol. 2003;28:417–80.CrossRefGoogle Scholar
  16. 16.
    Svensson LG, Batizy LH, Blackstone EH, Gillinov AM, Moon MC, D’Agostino RS, et al. Results of matching valve and root repair to aortic valve and root pathology. J Thorac Cardiovasc Surg. 2011;142:1491–8.CrossRefGoogle Scholar
  17. 17.
    Underwood MJ, El KG, Deronck D, Glineur D, Dion R. The aortic root: structure, function, and surgical reconstruction. Heart. 2000;83:376–80.CrossRefGoogle Scholar
  18. 18.
    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
  19. 19.
    Fedak PW, de Sa MP, Verma S, et al. Vascular matrix remodeling in patients with bicuspid aortic valve malformation: implications for aortic dilatation. J Thorac Cardiovasc Surg. 2003;126:797–806.CrossRefGoogle Scholar
  20. 20.
    Biner S, Rafique AM, Ray I, Cuk O, Siegel RJ, Tolstrup K. Aortopathy is prevalent in relatives of bicuspid aortic valve patients. J Am Coll Cardiol. 2009;53:2288–95.CrossRefGoogle Scholar
  21. 21.
    Silberbach M. Bicuspid aortic valve and thoracic aortic aneurysm: toward a unified theory. J Am Coll Cardiol. 2009;53:2296–7.CrossRefGoogle Scholar
  22. 22.
    Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J, et al. International registry of aortic dissection (IRAD). Characterizing the young patient with aortic dissection: results from the international registry of aortic dissection (IRAD). J Am Coll Cardiol. 2004;43:665–9.CrossRefGoogle Scholar
  23. 23.
    Roberts CS, Roberts WC. Dissection of the aorta associated with congenital malformation of the aortic valve. J Am Coll Cardiol. 1991;17:712–6.CrossRefGoogle Scholar
  24. 24.
    Nkomo VT, Enriquez-Sarano M, Ammash NM, Melton LJ 3rd, Bailey KR, Desjardins V, Horn RA, Tajik AJ. Bicuspid aortic valve associated with aortic dilatation: a community-based study. Arterioscler Thromb Vasc Biol. 2003;23:351–6.CrossRefGoogle Scholar
  25. 25.
    Freyberg MA1, Kaiser D, Graf R, Buttenbender J, Friedl P. Proatherogenic flow conditions initiate endothelial apoptosis via thrombospondin-1 and the integrin-associated protein. Biochem Biophys Res Commun. 2001;286:141–9.CrossRefGoogle Scholar
  26. 26.
    El Khoury G, Glineur D, Rubay J, Verhelst R, d’Acoz Y, Poncelet A, et al. Functional classification of aortic root/valve abnormalities and their correlation with etiologies and surgical procedures. Curr Opin Cardiol. 2005;20:115–21.CrossRefGoogle Scholar
  27. 27.
    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
  28. 28.
    Boodhwani M, de Kerchove L, Glineur D, Rubay J, Vanoverschelde JL, Noirhomme P, El Khoury G. Repair of regurgitant bicuspid aortic valves: a systematic approach. J Thorac Cardiovasc Surg. 2010;140:276–84.CrossRefGoogle Scholar
  29. 29.
    Navarra E, El Khoury G, Glineur D, Boodhwani M, Van Dyck M, Vanoverschelde JL, Noirhomme P, de Kerchove L. Effect of annulus dimension and annuloplasty on bicuspid aortic valve repair. Eur J Cardiothorac Surg. 2013;44:316–22.CrossRefGoogle Scholar
  30. 30.
    Schäfers HJ, Kunihara T, Fries P, Brittner B, Aicher D. Valve-preserving root replacement in bicuspid aortic valves. J Thorac Cardiovasc Surg. 2010;140:S36–40.CrossRefGoogle Scholar
  31. 31.
    Hammermeister K, Sethi GK, Henderson WG, Grover FL, Oprian C, Rahimtoola SH. Outcomes 15 years after valve replacement with a mechanical versus a bioprosthetic valve: final report of the veterans affairs randomized trial. J Am Coll Cardiol. 2000;36:1152–8.CrossRefGoogle Scholar
  32. 32.
    Svensson LG. Minimal-access “J” or “j” sternotomy for valvular, aortic, and coronary operations or reoperations. Ann Thorac Surg. 1997;64:1501–3.CrossRefGoogle Scholar
  33. 33.
    Trusler GA, Kazenelson G, Freedom RM, Williams WG, Rowe RD. Late results following repair of partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. J Thorac Cardiovasc Surg. 1980;79:776–81.PubMedGoogle Scholar
  34. 34.
    Frater RW. The prolapsing aortic cusp (experimental and clinical observations). Ann Thorac Surg. 1967;3:63–7.CrossRefGoogle Scholar
  35. 35.
    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.CrossRefGoogle Scholar
  36. 36.
    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.CrossRefGoogle Scholar
  37. 37.
    Elgamal MA, Hakimi M, Lyons JM, Walters HL 3rd. Rısk factors for failure of aortic valvuloplasty in aortic insufficiency with ventricular septal defect. Ann Thorac Surg. 1999;68:1350–5.CrossRefGoogle Scholar
  38. 38.
    Odim J, Laks H, Allada V, Child J, Wilson S, Gjertson D. Results of aortic valve-sparing and restoration with autologous pericardial leaflet extensions in congenital heart disease. Ann Thorac Surg. 2005;80:647–53.CrossRefGoogle Scholar
  39. 39.
    Toeg HD, Abessi O, Al-Atassi T, de Kerchove L, El-Khoury G, Labrosse M, Boodhwani M. Finding the ideal biomaterial for aortic valve repair with ex vivo porcine left heart simulator and finite element modeling. J Thorac Cardiovasc Surg. 2014;148:1739–45.CrossRefGoogle Scholar
  40. 40.
    Della Corte A, Body SC, Booher AM, Schaefers HJ, Milewski RK, Michelena HI, et al. International bicuspid aortic valve consortium (BAVCon) investigators. Surgical treatment of bicuspid aortic valve disease: knowledge gaps and research perspectives. J Thorac Cardiovasc Surg. 2014;147:1749–57.CrossRefGoogle Scholar
  41. 41.
    David TE, Feindel CM. An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta. J Thorac Cardiovasc Surg. 1992;103:617–21.PubMedGoogle Scholar
  42. 42.
    Sarsam MA, Yacoub M. Remodeling of the aortic valve anulus. J Thorac Cardiovasc Surg. 1993;105:435–8.PubMedGoogle Scholar
  43. 43.
    Schäfers H, Fries R, Langer F, Nikoloudakis N, Graeter T, Grundmann U. Valve-preserving replacement of the ascending aorta: remodeling versus reimplantation. J Thorac Cardiovasc Surg. 1998;116:990–6.CrossRefGoogle Scholar
  44. 44.
    van Son JA, Battellini R, Mierzwa M, Walther T, Autschbach R, Mohr FW. Aortic root reconstruction with preservation of native aortic valve and sinuses in aortic root dilatation with aortic regurgitation. J Thorac Cardiovasc Surg. 1999;117:1151–6.CrossRefGoogle Scholar
  45. 45.
    de Kerchove L, Boodhwani M, Glineur D, Vandyck M, Vanoverschelde JL, Noirhomme P, El Khoury G. 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
  46. 46.
    Frater RW. Aortic valve insufficiency due to aortic dilatation: correction by sinus rim adjustment. Circulation. 1986;74:I136–42.PubMedGoogle Scholar
  47. 47.
    Schafers HJ, Aicher D, Langer F, Lausberg HF. Preservation of the bicuspid aortic valve. Ann Thorac Surg. 2007;83:S740–5.CrossRefGoogle Scholar
  48. 48.
    Aicher D, Kunihara T, Abou IO, Brittner B, Graber S, Schafers HJ. Valve configuration determines long-term results after repair of the bicuspid aortic valve. Circulation. 2011;123:178–85.CrossRefGoogle Scholar
  49. 49.
    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.PubMedGoogle Scholar
  50. 50.
    Lansac E, Di Centa I, Bonnet N, Leprince P, Rama A, Acar C, Pavie A, Gandjbakhch I. Aortic prosthetic ring annuloplasty: a useful adjunct to a standardized aortic valve-sparing procedure? Eur J Cardiothorac Surg. 2006;29:537–44.CrossRefGoogle Scholar
  51. 51.
    Lansac E, Di Centa I, Varnous S, Rama A, Jault F, Duran CM, et al. External aortic annuloplasty ring for valve-sparing procedures. Ann Thorac Surg. 2005;79:356–8.CrossRefGoogle Scholar
  52. 52.
    Svensson LG, Longoria J, Kimmel WA, Nadolny E. Management of aortic valve disease during aortic surgery. Ann Thorac Surg. 2000;69:778–83.CrossRefGoogle Scholar
  53. 53.
    Hanke T, Charitos EI, Stierle U, Robinson D, Gorski A, Sievers HH, Misfeld M. Factors associated with the development of aortic valve regurgitation over time after two different techniques of valve-sparing aortic root surgery. J Thorac Cardiovasc Surg. 2009;137:314–9.CrossRefGoogle Scholar
  54. 54.
    Kari FA, Siepe M, Sievers HH, Beyersdorf F. Repair of the regurgitant bicuspid or tricuspid aortic valve: background, principles, and outcomes. Circulation. 2013;128:854–63.CrossRefGoogle Scholar
  55. 55.
    Jermihov PN, Jia L, Sacks MS, Gorman RC, Gorman JH, Chandran KB. Effect of geometry on the leaflet stresses in simulated models of congenital bicuspid aortic valves. Cardiovasc Eng Technol. 2011;2:48–56.CrossRefGoogle Scholar
  56. 56.
    Kunihara T, Aicher D, Rodionycheva S, Groesdonk HV, Langer F, Sata F, Schäfers HJ. Preoperative aortic root geometry and postoperative cusp configuration primarily determine long-term outcome after valve-preserving aortic root repair. J Thorac Cardiovasc Surg. 2012;143:1389–95.CrossRefGoogle Scholar
  57. 57.
    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.CrossRefGoogle Scholar
  58. 58.
    le Polain de Waroux JB, Pouleur AC, Robert A, Pasquet A, Gerber BL, Noirhomme P, El Khoury G, Vanoverschelde JL. Mechanisms of recurrent aortic regurgitation after aortic valve repair: predictive value of intraoperative transesophageal echocardiography. JACC Cardiovasc Imaging. 2009;2:931–9.CrossRefGoogle Scholar
  59. 59.
    Svensson LG, Deglurkar I, Ung J, Pettersson G, Gillinov AM, D’Agostino RS, Lytle BW. Aortic valve repair and root preservation by remodeling, reimplantation, and tailoring: technical aspects and early outcome. J Card Surg. 2007;22:473–9.CrossRefGoogle Scholar
  60. 60.
    Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE Jr, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology,American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons,and Society for Vascular Medicine. J Am Coll Cardiol. 2010;55:e27–e129.CrossRefGoogle Scholar
  61. 61.
    Svensson LG, Kim KH, Blackstone EH, Rajeswaran J, Gillinov AM, Mihaljevic T, et al. Bicuspid aortic valve surgery with proactive ascending aorta repair. J Thorac Cardiovasc Surg. 2011;142:622–9.CrossRefGoogle Scholar
  62. 62.
    Svensson LG, Adams DH, Bonow RO, Kouchoukos NT, Miller DC, O’Gara PT, et al. Aortic valve and ascending aorta guidelines for management and quality measures. Ann Thorac Surg. 2013;95:S1–66.CrossRefGoogle Scholar
  63. 63.
    Pettersson GB, Crucean AC, Savage R, Halley CM, Grimm RA, Svensson LG, et al. Toward predictable repair of regurgitant aortic valves: a systematic morphology-directed approach to bicommissural repair. J Am Coll Cardiol. 2008;52:40–9.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Cardiovascular SurgeryJohns Hopkins All Children’s Heart InstituteSt PetersburgUSA
  2. 2.Cardiac, Aortic and Endovascular SurgeryUniversity of British ColumbiaVancouverCanada
  3. 3.Cleveland ClinicClevelandUSA

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