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Bicuspid Aortic Valve

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Book cover Aortopathy

Abstract

Bicuspid aortic valve (BAV) has a prevalence of 0.5–1.39% in the general population. The prevalence of BAV in aortic dissection is 3.5–11.8%. Unfortunately, the incidence of aortic dissection in BAV remains unknown. The etiology of BAV is polygenetic, where environmental factors and unknown genetic factors seem to interact to cause BAV. In some instances, chromosomal aberrations or defined gene defects cause BAV.

Congenital BAV must be distinguished from acquired BAV. Congenital aortic valve malformations differ by number of cusps, ranging from one to five. BAV cusps can be subclassified according to patterns of calcification, severity of calcification, presence of a raphe, and fusion of cusps. We tend to perceive BAV as an isolated congenital heart defect. However, we identified 20 well-defined syndromic, complex, or isolated congenital heart defects that are associated with BAV disease, some of which are apparently quite frequent.

BAV aortopathy can be classified according to presence and type of aortic valve dysfunction, shape of the proximal aorta, aortic arch involvement, and coexistence with coarctation of the aorta.

Factors that may increase the risk for aneurysmal formation, aortic rupture, or dissection in BAV comprise aortic valve characteristics, comorbidities of BAV, and behavioral factors. Candidates for biomarkers of BAV aortopathy comprise a family history with early dissection or death, increased aortic growth rates, proximal aortic shape, aortic stiffness and aortic elasticity markers, aortic wall shear stress, endothelial dysfunction, and serological biomarkers.

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Abbreviations

AOA:

Aortic arch

ASC:

Ascending aorta

AVA:

Aortic valve annulus (anatomical ventriculo-arterial junction)

AVR:

Aortic valve replacement

BAV:

Bicuspid aortic valve

BAV-COA:

Coexistence of bicuspid aortic valve and coarctation of the aorta

BAV-I:

BAV with predominant insufficiency

BAV-LN:

BAV with fusion of the left and noncoronary cusp

BAV-MO:

BAV morphotype

BAV-RI:

BAV with balanced stenosis and insufficiency

BAV-RL:

BAV with fusion of the right and left coronary cusp

BAV-RN:

BAV with fusion of the right and noncoronary cusp

BAV-S:

BAV with predominant stenosis

CHD:

Congenital heart defect

COA:

Coarctation of the aorta

DESC:

Descending thoracic aorta

HTN:

Arterial hypertension

SOV:

Sinus of valsalva

STJ:

Sinotubular junction

TAV:

Tricuspid aortic valve

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  1. Department of Cardiology/Angiology, Centre of Cardiology and Cardiovascular Surgery, University Hospital Hamburg – Eppendorf, Hamburg, Germany

    Yskert von Kodolitsch M.D., M.B.A.

  2. Klinik für Kinderkardiologie und angeborene Herzfehler, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, D-80636, München, Germany

    Harald Kaemmerer M.D.

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  1. Cardiovascular Center, St Luke’s International Hospital, Chuo-ku, Tokyo, Japan

    Koichiro Niwa

  2. ACHD Unit, German Heart Centre Munich, Munich, Bayern, Germany

    Harald Kaemmerer

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von Kodolitsch, Y., Kaemmerer, H. (2017). Bicuspid Aortic Valve. In: Niwa, K., Kaemmerer, H. (eds) Aortopathy. Springer, Tokyo. https://doi.org/10.1007/978-4-431-56071-5_15

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  • Online ISBN: 978-4-431-56071-5

  • eBook Packages: MedicineMedicine (R0)

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