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Bicuspid Aortic Valve: Morphology, Clinic, Diagnosis, Complications

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Bicuspid aortic valve refers to common (0.5-2% of the population) congenital heart defects that are asymptomatic throughout life, with valve dysfunction and/or aortopathy (pathia- from Greek pathos disease), manifested by expansion, aneurysm or dissection of the vessel. The pathogenesis of the formation of a bicuspid valve is unknown, a genetic component is noted, since the defect develops as sporadic, familial, in combination with other congenital heart defects and with hereditary connective tissue disorders. Morphogenetic studies suggest that different phenotypes of bicuspid aortic valve can be considered as etiologically different diseases, with valve dysfunction or valve dysfunction and aortopathy. Aortic lesion is characterized by phenotypic heterogeneity due to genetic or hemodynamic features. Researchers are discussing the relationship between the phenotype of the bicuspid aortic valve and aortopathy to predict the course of the disease and select the optimal surgical treatment technique. Diagnosis of heart disease is based on the results of an echocardiographic study, magnetic resonance imaging. Surgical treatment is performed for significant hemodynamic disturbances resulting from insufficiency or stenosis of the aortic valve, in cases of infective endocarditis, the risk of which is high, with aneurysm or aortic dissection.

About the Author

Y. L. Trysvetava
Belarusian State Medical University

Yauheniya L. Trysvetava


eLibrary SPIN: 9443-2354


1. Marelli A.J., Ionescu-Ittu R., Makie A.S. et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014;130(9):749-56. DOI:10.1161/CIRCULATIONAHA.113.008396.

2. Michelena H.I., Prakash S.K., della Corte A., et al. Bicuspid aortic valve: Identifying knowledge gaps and rising to the challenge from the international bicuspid aortic valve consortium (BAVCon). Circulation. 2014;129(5):2691-704. DOI:10.1161/CIRCULATIONAHA.113.007851.

3. Hoffman J.I., Kaplan S., Liberthson R.R. Prevalence of congenital heart disease. Am Heart J. 2004;147(3):425-39. DOI:10.1016/j.ahj.2003.05.003.

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(3):513-15. DOI:10.1016/j.ahj.2004.10.036.

5. Kong W.K.F., Regeer M., Ng A.C., et al. Sex Differences in Phenotypes of Bicuspid Aortic Valve and Aortopathy: Insights From a Large Multicenter, International Registry. Circ Cardiovasc Imaging. 2017;10(3). pii: e005155. DOI:10.1161/CIRCIMAGING.116.005155.

6. Restivo A., Piacentini G., Placidi S., et al. Cardiac outflow tract: a review of some embryogenetic aspects of the conotruncal region of the heart. Anat Rec A Discov Mol Cell Evol Biol. 2006;288(9):936- 43. DOI:10.1002/ar.a.20367.

7. Mikhailov S.S. Clinical anatomy of the heart. Moscow: Medicine; 1987 (In Russ.)

8. Sievers H.H., Schmidtke C.A. Classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg. 2007;133(5):1226-33. DOI:10.1016/j.jtcvs.2007.01.039.

9. Harris C., Croce B., Munkholm-Larsen S. Bicuspid aortic valve. Ann Cardiothorac Surg. 2017;6(6):721. DOI:10.21037/acs.2017.11.07.

10. Siu S.C., Silversides C.K. Bicuspid aortic valve disease. J Am Coll Cardiol. 2010;55(25):2789-800. DOI:10.1016/j.jacc.2009.12.068.

11. Malčić I., Grgat J., Kniewald H., et al. Bicuspid aortic valve and left ventricular outflow tract defects in children - syndrome of bicuspid aortopathy? Lijec Vjesn. 2015;137(9-10):267-75.

12. Braverman A.C., Beardslee M.A. The bicuspid aortic valve. In: Otto C.M., Bonow R.O., eds. Valvular heart disease: a companion to Braunwald's heart disease. 3rd ed. Philadelphia: Saunders/Elsevier; 2009: 169-86.

13. Freeze S.L., Landis B.J., Ware S.M., et al. Bicuspid Aortic Valve: a Review with Recommendations for Genetic Counseling. J Genet Couns. 2016;25(6):1171-78. DOI:10.1007/s10897-016-0002-6.

14. Fernández B., Duran A.C., Fernández-Gallego T., et al. Bicuspid aortic valves with different spatial orientations of the leaflets are distinct etiological entities. J Am Coll Cardiol. 2009;54(24):2312- 18. DOI:10.1016/j.jacc.2009.07.044.

15. Shan S.Y., Higgins A., Desay M.Y. Bicuspid aortic valve: Basics and beyond. Cleve Clin J Med. 2018;85(10):779-84. DOI:10.3949/ccjm.85a.17069.

16. Girdauskas E., Routman M., Kusntrim D., et al. Morphologic and Functional Markers of Aortopathy in Patients with Bicuspid Aortic Valve Insufficiency Versus Stenosis. Ann Thorac Surg. 2017;103(1):49- 57. DOI:10.1016/j.athoracsur.2016.05.085.

17. Girdauskas E., Rouman M., Disha K., et al. Aortic dissection after previous aortic valve replacement for bicuspid aortic valve disease. J Am Coll Cardiol. 2015;66(12):1409-11. DOI:10.1016/j.jacc.2015.07.022.

18. Verma S., Siu S.C. Aortic dilatation in patients with bicuspid aortic valve. N Engl J Med. 2014;370(20):1920-9. DOI:10.1056/NEJMra1207059.

19. Della Corte A., Bancone C., Dialetto G., et al. The ascending aorta with bicuspid aortic valve: a phenotypic classification with potential prognostic significance. Eur J Cardiothorac Surg. 2014;46(2):240- 7. discussion 247. DOI:10.1093/ejcts/ezt621.

20. 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(3):e118-e124. DOI:10.1016/j.ejcts.2011.04.014.

21. Fazel S.S., Mallidi H.R., Lee R.S., 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(4):901- 7. DOI:10.1016/j.jtcvs.2008.01.022.

22. Oladokun D., Patterson B.O., Sobocinski J., et al., Systematic review of the growth rates and influencing factors in thoracic aortic aneurysms. Eur J Vasc Endovasc Surg. 2016;51(5):674-81. DOI:10.1016/j.ejvs.2016.01.017.

23. Detaint D., Michelena H.I., Nkomo V.T., et al. Aortic dilatation patterns and rates in adults with bicuspid aortic valves: A comparative study with Marfan syndrome and degenerative aortopathy. Heart. 2014;100(2):126-34. DOI:10.1136/heartjnl-2013-304920.

24. Sievers H.H., Stierle U., Hachmann R.M., et al. New insights in the association between bicuspid aortic valve phenotype, aortic configuration and valve haemodynamics. Eur J Cardiothorac Surg. 2016;49(2):439-46. DOI:10.1093/ejcts/ezv087.

25. Etz C.D., von Aspern K., Hoyer A., et al. Acute type A aortic dissection: characteristics and outcomes comparing patients with bicuspid versus tricuspid aortic valve. Eur J Cardiothorac Surg. 2015;48(1):142- 50. DOI:10.1093/ejcts/ezu388.

26. Miskowiec D., Lipiec P., Szymczyk E., et al. Bicuspid aortic valve morphology and its impact on aortic diameters-A systematic review with meta-analysis and meta-regression. Echocardiography. 2018;35(5):667-77. DOI:10.1111/echo.13818.

27. Borger M.A., Fedak P.W.M., Stephens E.H., et al. The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valverelated aortopathy: Full online-only version. J Thorac Cardiovasc Surg. 2018;156(2):e41-e74. DOI:10.1016/jtcvs.2018.02.115.

28. Dzemeshkevich S.L., Stevenson L.U., Alexi-Meskhishvili V.V. Diseases of the aortic valve (function, diagnosis, treatment). Moscow: GEOTAR-MED; 2004 (In Russ.)

29. Roberts W.C., Ko J.M. 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(7):920-5. DOI:10.1161/01.CIR.0000155623748408.C5.

30. Davies R.R., Kaple R.K., Mandapati D., et al. Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Ann Thorac Surg. 2007;83(4):1338-44. DOI:10.1016/j.athoracsur.2006.10.074.

31. Wilton E., Bland M., Thompson M., et al. Matrix metalloproteinase expression in the ascending aorta and aortic valve. Interact Cardiovasc Thorac Surg. 2008;7(1):37-40. DOI:10.1510/icvts.2007.163311.

32. Wang Y., Wu B., Dong L., et al. Circulating matrix metalloproteinase patterns in association with aortic dilatation in bicuspid aortic valve patients with isolated severe aortic stenosis. Heart Vessels. 2016;31(2):189-97. DOI:10.1007/s00380-014-0593-5.

33. Sophocleous E., Milano E.G., Pontecorboli G. et al. Enlightening the Association between Bicuspid Aortic Valve and Aortopathy. J Cardiovasc Dev Dis. 2018;5(2): pii:E21. DOI:10.3390/jcdd5020021.

34. Sabet H.Y., Edwards W.D., Tazelaar H.D., 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(1):14-26. DOI:10.4065/74.1.14.

35. Boonyasirinant T., Rajiah P., Flamm S.D. Abnormal aortic stiffness in patients with bicuspid aortic valve: phenotypic variation determined by magnetic resonance imaging. Int J Cardiovasc Imaging. 2018; 35(1):133-41. DOI:10.1007/s10554-018-1433-y.

36. Singh A., Horsfield M.A., Bekele S., et al. Aortic stiffness in aortic stenosis assessed by cardiovascular MRI: a comparison between bicuspid and tricuspid valves. Eur Radiol. 2019;29(5):2340-49. DOI:10.1007/s00330-018-5775-6.

37. Evangelista A., Gallego P., Calvo-Iglesias F. et al. Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease. Heart. 2018;104(7):566-73. DOI:10.1136/heartjnl2017-311560.

38. Tribouilloy C., Rusinaru D., Sorel C., et al. Clinical characteristics and outcome of infective endocarditis in adults with bicuspid aortic valves: a multicentre observational study. Heart. 2010;96(21):1723- 29. DOI:10.1136/hrt.2009.189050.

39. Chan K.L., Stinson W.A., Veinot J.P. Reliability of transthoracic echocardiography in the assessment of aortic valve morphology: pathological correlation in 178 patients. Can J Cardiol. 1999;15(1):48- 52.

40. Donato Aquaro G., Ait-Ali L., Basso M.L., et al. Elastic properties of aortic wall in patients with bicuspid aortic valve by magnetic resonance imaging. Am J Cardiol. 2011;108(1):81-7. DOI:10.1016/j.amjcard.2011.03.005.

41. Egbe A.C., Padanq R., Brown R.D., et al. Prevalence and predictors of intracranial aneurysms in patients with bicuspid aortic valve. Heart. 2017;103(19):1508-14. DOI:10.1136/heartjnl-2016-311076.

For citation:

Trysvetava Y.L. Bicuspid Aortic Valve: Morphology, Clinic, Diagnosis, Complications. Rational Pharmacotherapy in Cardiology. 2021;17(1):111-118. (In Russ.)

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