Cited by 1,028Aortic Disease and Treatment ApproachesCardiac Valve Diseases and TreatmentsCongenital Heart Disease Studies

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Bicuspid Aortic Valve Disease

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Siu, Samuel C., Silversides, Candice K.·2010·Journal of the American College of Cardiology

DOI 10.1016/j.jacc.2009.12.068

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Abstract

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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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)
DOI: 10.1016/j.jacc.2009.12.068
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: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
www.elsevier.com/open-access/userlicense/1.0/ (vor, 1178 d)
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

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