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The Aortopathy of Bicuspid Aortic Valves

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Controversies in Aortic Dissection and Aneurysmal Disease

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Abstract

Bicuspid aortic valve (BAV) disease is a common congenital malformation associated with significant morbidity and mortality, mostly related to valvular dysfunction. BAV has been associated with a dilated proximal aorta and a risk of dissection and rupture. Uncertainty remains about whether this aortopathy is caused by molecular dysregulation during embryonic development, or whether aortic root dilation is a function of abnormal hemodynamics. This has therapeutic ramifications especially since the optimal timing of aortic root replacement in BAV is not known and the etiology of aortic root dilation will be instrumental in devising a definitive answer to this problem. In this chapter, we will discuss the genetics and molecular biology of BAV, especially as they pertain to aortic root dilation. We will also present the two competing theories of BAV aortopathy. Attention is then directed to current guidelines for surveillance and treatment of BAV aortopathy.

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  1. Department of Medicine, Boston Medical Center, 88 East Newton Street, Boston, MA, 02118, USA

    Omar K. Siddiqi MD & Michael D. Klein MD

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  1. University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, United Kingdom

    Robert S. Bonser

  2. University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, United Kingdom

    Domenico Pagano

  3. Thoracic and Cardiovascular Surgery Depts., Hannover Medical School, Hannover, Germany

    Axel Haverich

  4. University Hospitals Birmingham, Queen Elizabeth Hospital & Centre, Birmingham, United Kingdom

    Jorge Mascaro

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Siddiqi, O.K., Klein, M.D. (2014). The Aortopathy of Bicuspid Aortic Valves. In: Bonser, R., Pagano, D., Haverich, A., Mascaro, J. (eds) Controversies in Aortic Dissection and Aneurysmal Disease. Springer, London. https://doi.org/10.1007/978-1-4471-5622-2_3

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