Unicuspid Aortic Valve: A Review of Literature

Unicuspid aortic valve (UAV) is a rare congenital heart disease that leads to aortic valve dysfunction, most commonly stenosis, but also regurgitation. It typically presents in the third to fourth decade of life. Because of its rarity, UAV is less well-characterized than other congenital valve disorders, such as the bicuspid aortic valve (BAV). Furthermore, its similarities to BAV often lead to misdiagnosis, making accurate differentiation critical for management and prognosis. This article reviews the literature on this rare condition regarding its epidemiology, embryology, genetics, clinical presentation, diagnosis, and management.

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Journal
Journal of the Heart Valve Society
Published
2026-09-04
DOI
https://doi.org/10.1177/30494826261484712
Primary Topic
Aortic Disease and Treatment Approaches
Type
article
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Unicuspid Aortic Valve: A Review of Literature

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Journal of the Heart Valve Society
Aortic Disease and Treatment Approaches
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Unicuspid Aortic Valve: A Review of Literature

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article en

Abstract

Unicuspid aortic valve (UAV) is a rare congenital heart disease that leads to aortic valve dysfunction, most commonly stenosis, but also regurgitation. It typically presents in the third to fourth decade of life. Because of its rarity, UAV is less well-characterized than other congenital valve disorders, such as the bicuspid aortic valve (BAV). Furthermore, its similarities to BAV often lead to misdiagnosis, making accurate differentiation critical for management and prognosis. This article reviews the literature on this rare condition regarding its epidemiology, embryology, genetics, clinical presentation, diagnosis, and management.

Journal of the Heart Valve Society
Komfo Anokye Teaching Hospital (GH), Kwame Nkrumah University of Science and Technology (GH), Kwame Nkrumah University (ZM)
Good health and well-being
Openalex Percentile: Top 11%
Aortic Disease and Treatment Approaches
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Unicuspid Aortic Valve: A Review of Literature — Collins Kokuro, Isaac Kofi Owusu, et al. · Journal of the Heart Valve Society (2026) | TGRS Research Map | TGRS