Bicuspid aortic valve – ethiopathogenesis and natural history

Czech version

Authors: M. Brtko ¹; J. Vojáček ¹; M. Tuna ¹; P. Telekes ²
Authors‘ workplace: Kardiocentrum, Kardiochirurgická klinika Lékařské fakulty UK a FN Hradec Králové, přednosta doc. MUDr. Jan Harrer, CSc. ¹; Kardiocentrum, Krajská nemocnice Liberec, přednosta MUDr. Rostislav Polášek ²
Published in: Vnitř Lék 2011; 57(2): 176-182
Category: Reviews

Overview

Bicuspid aortic valve is a disease with autosomal dominant inheritance with small penetration. The population of patients with bicuspid aortic valve is heterogenous. In the absence of dilatation of ascending aorta, valvular lesion or degenerative changes of valve leaflets patients are considered to belong to the low risk population. On the other hand patients with the above mentioned characteristics compose a high risk group. Dilatation of the ascending aorta in patients with bicuspid aortic valve has a progressive feature and continues even after replacement of the aortic valve. With progression of aortic dilatation the risk of aortic dissection and rupture increases. The main reasons for aortic dilatation are changes in quality of aortic wall and possibly mechanical stress of aortic wall during asymmetrical and turbulent flow. The progression of aortic dilatation or aortic valve disease is not essentialy influenced by farmacologic treatment. Surgery is the treatment of choice. Physiology and pathophysiology of bicuspid and tricuspid aortic valves, prevalence of bicuspid aortic valve in general population and in the group of patients operated on for aortic valve disease, phenotypes of bicuspid aortic valve and follow-up of asymptomatic patients with normal bicuspid valve are rewied in this article. Indications for operation of dilated ascending aorta according to american, european and czech guidelines are discussed.

Key words:
bicuspid aortic valve – ethiopathogenesis – dilatation of ascending aorta – dissection – rupture

Sources

1. 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: 177–185.

2. 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.

3. Cripe L, Andelfinger G, Martin LJ et al. Bicuspid aortic valve is heritable. J Am Coll Cardiol 2004; 44: 138–143.

4. Vallely MP, Semsarian C, Bannon PG. Management of the ascending aorta in patients with bicuspid aortic valve disease. Heart Lung Circ 2008; 17: 357–363.

5. Bauer M, Gliech V, Siniawski H et al. 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 2006; 15: 594–600.

6. Nistri S, Basso C, Marzari C et al. Frequency of bicuspid aortic valve in young male conscripts by echocardiogram. Am J Cardiol 2005; 96: 718–721.

7. Sonoda M, Takenaka K, Uno K et al. A larger aortic annulus causes aortic regurgitation and a smaller aortic annulus causes aortic stenosis in bicuspid aortic valve. Echocardiography 2008; 25: 242–248.

8. Schaefer BM, Lewin MB, Stout KK et al. Usefulness of bicuspid aortic valve phenotype to predict elastic properties of the ascending aorta. Am J Cardiol 2007; 99: 686–690.

9. Schaefer BM, Lewin MB, Stout KK et al. The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape. Heart 2008; 94: 1634–1638.

10. Fazel SS, Mallidi HR, Lee RS et al. The aortopathy of bicuspid aortic valve disease has distinctive patterns and usually involves the transverse aortic arch. J Thorac Cardiovasc Surg 2008; 135: 901–907.

11. Collins MJ, Butany J, Borger MA et al. Implications of a congenitally abnormal valve: a study of 1025 consecutively excised aortic valves. J Clin Pathol 2008; 61: 530–536.

12. Bauer M, Bauer U, Siniawski H et al. Differences in clinical manifestations in patients with bicuspid and tricuspid aortic valves undergoing surgery of the aortic valve and/or ascending aorta. Thorac Cardiovasc Surg 2007; 55: 485–490.

13. 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: 2776–2784.

14. Nistri S, Sorbo MD, Marin M et al. Aortic root dilatation in young men with normally functioning bicuspid aortic valves. Heart 1999; 82: 19–22.

15. Keane MG, Wiegers SE, Plappert T et al. Bicuspid aortic valves are associated with aortic dilatation out of proportion to coexistent valvular lesions. Circulation 200; 102 (19 Suppl 3): III35–III39.

16. 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.

17. 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.

18. Nistri S, Sorbo MD, Basso C et al. Bicuspid aortic valve: abnormal aortic elastic properties. J Heart Valve Dis 2002; 11: 369–374.

19. Nistri S, Grande-Allen J, Noale M et al. Aortic elasticity and size in bicuspid aortic valve syndrome. Eur Heart J 2008; 29: 472–479.

20. Grotenhuis HB, Ottenkamp J, Westenberg JJ et al. Reduced aortic elasticity and dilatation are associated with aortic regurgitation and left ventricular hypertrophy in nonstenotic bicuspid aortic valve patients. J Am Coll Cardiol 2007; 49: 1660–1665.

21. Della Corte A, Romano G, Tizzano F et al. Echocardiographic anatomy of ascending aorta dilatation: correlations with aortic valve morphology and function. Int J Cardiol 2006; 113: 320–326.

22. 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: 397–405.

23. Linhartová K, Beránek V, Šefrna F et al. Aortic stenosis severity is not a risk factor for poststenotic dilatation of the ascending aorta. Circ J 2007; 71: 84–88.

24. Linhartová K, Beránek V, Šefrna F et al. Závisí poststenotická dilatace kořene aorty na významnosti aortální stenózy? Cor Vasa 2005; 47: 183–186.

25. La Canna G, Ficarra E, Tsagalau E et al. Progression rate of ascending aortic dilation in patients with normally functioning bicuspid and tricuspid aortic valves. Am J Cardiol 2006; 98: 249–253.

26. Davies RR, Kaple RK, Mandapati D et al. Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Ann Thorac Surg 2007; 83: 1338–1344.

27. Goland S, Czer LS, De Robertis MA et al. 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: 931–937.

28. 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.

29. Davies RR, Goldstein LJ, Coady MA et al. Yearly rupture or dissection rates for thoracic aortic aneurysms: simple prediction based on size. Ann Thorac Surg 2002; 73: 17–28.

30. Bonow RO, Carabello BA, Chatterjee 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 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–e148.

31. Vahanian A, Baumgartner H, Bax J et al. Guidelines on the management of valvular heart disease: The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology. Eur Heart J 2007; 28: 230–268

32. Popelová J, Benešová M, Brtko M et al. Doporučené postupy pro diagnostiku a léčbu chlopenních srdečních vad v dospělosti. Cor Vasa 2007; 49: K195–K234.