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Bicuspid Aortic Valve Disease
Open accessAbstract
Bicuspid aortic valve (BAV) disease is the most common congenital cardiac defect. While the BAV can be found in isolation, it is often associated with other congenital cardiac lesions. The most frequent associated finding is dilation of the proximal ascending aorta secondary to abnormalities of the aortic media. Changes in the aortic media are present independent of whether the valve is functionally normal, stenotic, or incompetent. Although symptoms often manifest in adulthood, there is a wide spectrum of presentations ranging from severe disease detected in utero to asymptomatic disease in old age. Complications can include aortic valve stenosis or incompetence, endocarditis, aortic aneurysm formation, and aortic dissection. Despite the potential complications, 2 large contemporary series have demonstrated that life expectancy in adults with BAV disease is not shortened when compared with the general population. Because BAV is a disease of both the valve and the aorta, surgical decision making is more complicated, and many undergoing aortic valve replacement will also need aortic root surgery. With or without surgery, patients with BAV require continued surveillance. Recent studies have improved our understanding of the genetics, the pathobiology, and the clinical course of the disease, but questions are still unanswered. In the future, medical treatment strategies and timing of interventions will likely be refined. This review summarizes our current understanding of the pathology, genetics, and clinical aspects of BAV disease with a focus on BAV disease in adulthood.
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“Male sex is recognized as a risk factor for CAVS, partly due to the higher prevalence of bicuspid aortic valve (BAV) in men, with a male-to-female ratio of 3:1 5 .”
From Sex-specific gene expression signatures of calcific aortic valve stenosis in a large collection of human tissues (2026) · cites this paper as [5]
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Metadata
- Authors
- Siu, S. C., & Silversides, C. K.
- Affiliations
- Western University (CA)
- University Health Network (CA)
- Corresponding author
- Siu, Samuel C.
- ORCID
- Siu, Samuel C.
- Venue
- Journal of the American College of Cardiology
- Publisher
- Elsevier BV
- Published
- 2010-06
- Volume
- 55
- Issue
- 25
- Pages
- 2789-2800
- ISSN
- 0735-1097 (ISSN-L)
- OpenAlex
- W2121967184
- Cited by
- 1,028
- References
- 143
- Citations by year
- 2026: 37 · 2025: 62 · 2024: 59 · 2023: 85 · 2022: 68 · 2021: 100 · 2020: 71 · 2019: 61 · 2018: 63 · 2017: 79 · 2016: 69 · 2015: 67 · 2014: 64 · 2013: 49 · 2012: 57
- FWCI
- 7.88
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of the American College of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- Scholarship@Western (Western University) (submittedVersion)
- Funders
- Heart and Stroke Foundation of Canada (NA 5927, NA 5662, T6085)
- Canadian Institutes of Health Research (53030, 93722)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Aortic Disease and Treatment Approaches
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Valve Diseases and Treatments
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Congenital Heart Disease Studies
- Keywords
- bicuspid aortic valve, congenital heart disease, aortic root dilatation, aortic stenosis, aortic incompetence, infective endocarditis, aortic aneurysm, aortic dissection, aortic valve replacement, aortic root surgery, life expectancy, pathobiology, clinical course, surveillance (medicine), medical treatment, intervention timing
- MeSH terms
- Adult, Aortic Valve, Aortic Valve Insufficiency, Aortic Valve Stenosis, Cause of Death, Combined Modality Therapy, Female, Heart Defects, Congenital, Humans, Magnetic Resonance Imaging, Male, Mitral Valve, Prognosis, Severity of Illness Index, Survival Rate, Echocardiography, Transesophageal, Risk Assessment, Heart Valve Prosthesis Implantation, Young Adult
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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