Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2022 Mar 31;12(4):518.
doi: 10.3390/life12040518.

Point on the Aortic Bicuspid Valve

Chloé Bernard  1   2 , Marie Catherine Morgant  1 , David Guillier  1 , Nicolas Cheynel  1   3 , Olivier Bouchot  2
Affiliations
Review

Point on the Aortic Bicuspid Valve

Chloé Bernard et al. Life (Basel). .

Abstract

Background-Bicuspid aortic valve (BAV) disease is the most prevalent congenital heart disease in the world. Knowledge about its subtypes origin, development, and evolution is poor despite the frequency and the potential gravity of this condition. Its prognosis mostly depends on the risk of aortic aneurysm development with an increased risk of aortic dissection. Aims-This review aims to describe this complex pathology in way to improve the bicuspid patients' management. Study design-We reviewed the literature with MEDLINE and EMBASE databases using MeSH terms such as "bicuspid aortic valve", "ascending aorta", and "bicuspid classification". Results-There are various classifications. They depend on the criteria chosen by the authors to differentiate subtypes. Those criteria can be the number and position of the raphes, the cusps, the commissures, or their arrangements regarding coronary ostia. Sievers' classification is the reference. The phenotypic description of embryology revealed that all subtypes of BAV are the results of different embryological pathogenesis, and therefore, should be considered as distinct conditions. Their common development towards aortic dilatation is explained by the aortic media's pathological histology with cystic medial necrosis. At the opposite, BAV seems to display a profound genetic heterogeneity with both sporadic and familial forms. BAV can be even isolated or combined with other congenital malformations. Conclusions-All those characteristics make this pathology a highly complex condition that needs further genetic, embryological, and hemodynamic explorations to complete its well described anatomy.

Keywords: Siever’s classification; aortic aneurism; bicuspid aortic valve; genetics; histology.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
BAV classification, according to Sievers.
Figure 2
Figure 2
Bicuspid aortic valve subtypes anatomy and distribution.
Figure 3
Figure 3
Embryology of the aortic valve.
See this image and copyright information in PMC

References

    1. Hoffman J.I., Kaplan S. The incidence of congenital heart disease. J. Am. Coll. Cardiol. 2002;39:1890–1900. doi: 10.1016/S0735-1097(02)01886-7. - DOI - PubMed
    1. Fedak P., Verma S., David T.E., Leask R., Weisel R.D., Butany J. Clinical and Pathophysiological Implications of a Bicuspid Aortic Valve. Circulation. 2002;106:900–904. doi: 10.1161/01.CIR.0000027905.26586.E8. - DOI - PubMed
    1. Schaefer B.M., Lewin M.B., Stout K.K., Gill E., Prueitt A., Byers P.H., Otto C.M. The bicuspid aortic valve: An integrated phenotypic classification of leaflet morphology and aortic root shape. Heart. 2008;94:1634–1638. doi: 10.1136/hrt.2007.132092. - DOI - PubMed
    1. Garg V. Molecular genetics of aortic valve disease. Curr. Opin. Cardiol. 2006;21:180–184. doi: 10.1097/01.hco.0000221578.18254.70. - DOI - PubMed
    1. Debl K., Djavidani B., Buchner S., Poschenrieder F., Schmid F.-X., Kobuch R., Feuerbach S., Riegger G., Luchner A. Dilatation of the ascending aorta in bicuspid aortic valve disease: A magnetic resonance imaging study. Clin. Res. Cardiol. 2008;98:114–120. doi: 10.1007/s00392-008-0731-0. - DOI - PubMed

LinkOut - more resources