Cited by 442Cardiac Valve Diseases and TreatmentsAortic Disease and Treatment ApproachesConnective tissue disorders research

Bicuspid Aortic Valve: Identifying Knowledge Gaps and Rising to the Challenge From the International Bicuspid Aortic Valve Consortium (BAVCon)

Open access
Michelena, Hector I., Prakash, Siddharth K., Della Corte, Alessandro, Bissell, Malenka M., Anavekar, Nandan, Mathieu, Patrick, Bossé, Yohan, Limongelli, Giuseppe +10 more·2014·Circulation

Abstract

Since its estimated first description >500 years ago by Leonardo da Vinci,2 the bicuspid aortic valve (BAV) has progressively built a reputation; initially, as a curious valvular phenotype with a tendency to develop obstruction and insufficiency. In more contemporary times, however, the BAV is recognized as underlying almost 50% of isolated severe aortic stenosis cases requiring surgery,3 and has been extensively associated with ominous outcomes such as bacterial endocarditis and aortic dissection.4 These associations, coupled with the high prevalence of BAV in humans,5 have prompted investigative efforts into the condition, which although insightful, have generated more questions than answers. This review describes our current knowledge of BAV, but, more importantly, it highlights knowledge gaps and areas where basic and clinical research is warranted. Our review has 2 sections. The first section outlines the multifaceted challenge of BAV, our current understanding of the condition, and barriers that may hamper the advancement of the science. The second section proposes a roadmap to discovery based on current imaging, molecular biology, and genetic tools, recognizing their advantages and limitations.

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Crossref · 10.1161/circulationaha.113.007851

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  • Bicuspid aortic valves have two functional leaflets often of unequal size and result from incomplete separation of commissures during embryonic development.

    “Bicuspid aortic valves have two functional leaflets often of unequal size. This abnormality results from incomplete separation of commissures during embryonic development8.”

    From Calcific aortic stenosis (2016) · cites this paper as [8]

    The section “A Valvuloaortopathy With Varied Phenotypes and Unpredictable Outcomes” describes two aortic cusps that are usually asymmetrical, supporting the description of the leaflets. The “Pathobiology” section discusses embryological development but does not say that incomplete separation of commissures causes bicuspid aortic valves; that causal explanation, to which the citation is attached, is not supported by our copy. (LLM, conf=0.86)

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Research works printed but not linked: 1

  1. [1]Einstein A On the method of theoretical physics: The Herbert Spencer lecture, delivered at Oxford 10 June 1933 Oxford University Press 1933

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Authors
Michelena, H. I., Prakash, S. K., Della Corte, A., Bissell, M. M., Anavekar, N., Mathieu, P., Bossé, Y., Limongelli, G., Bossone, E., Benson, D. W., Lancellotti, P., Isselbacher, E. M., Enriquez-Sarano, M., Sundt, T. M., Pibarot, P., Evangelista, A., Milewicz, D. M., & Body, S. C.
Affiliations
  • From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de...
  • Mayo Clinic (US)
  • Children's Hospital of Wisconsin (US)
Venue
Circulation
Published
2014-06-24
Volume
129
Issue
25
Pages
2691-2704
ISSN
  • 0009-7322 (ISSN-L)
  • 1524-4539
OpenAlex
W2023616993
Cited by
442
References
214
Citations by year
2026: 28 · 2025: 27 · 2024: 32 · 2023: 36 · 2022: 46 · 2021: 59 · 2020: 39 · 2019: 32 · 2018: 35 · 2017: 44 · 2016: 35 · 2015: 17 · 2014: 8 · 2013: 1 · 2012: 3
FWCI
23.82
Type
journal article
Language
en
Open access
Locations
  • Circulation (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
  • Corpus Université Laval (Université Laval) (submittedVersion)
  • Archivio Istituzionale della Ricerca - Università degli Studi della Campania "Luigi Vanvitelli" (submittedVersion)
  • Open Repository and Bibliography (University of Liège) (submittedVersion)
Funders
  • British Heart Foundation (FS/10/43/28415)
  • National Institutes of Health (UL1TR000371, UL1TR000170)
  • National Heart, Lung, and Blood Institute (R01HL114823)
  • National Center for Advancing Translational Sciences
Topics
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Valve Diseases and Treatments
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
Keywords
bicuspid aortic valve, aortic stenosis, aortic valve obstruction, aortic regurgitation, bacterial endocarditis, aortic dissection, congenital heart disease, aortic valve imaging, genetic susceptibility, research gaps
MeSH terms
Bicuspid Aortic Valve Disease, Aortic Valve, Diagnostic Imaging, Cardiac Surgical Procedures, Heart Valve Diseases, Humans, Health Knowledge, Attitudes, Practice

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