Key result
Unicuspid aortic valve requires accurate differentiation from BAV to guide proper management.
Why the study?
Unicuspid aortic valve is rare and less well-characterized than other congenital valve disorders, and its similarities to bicuspid aortic valve often lead to misdiagnosis.
This review summarizes the epidemiology, embryology, genetics, clinical presentation, diagnosis, and management of the rare unicuspid aortic valve.
Answers are grounded in this paper and cite their sources.
Precise imaging is essential to differentiate UAV from BAV and guide surgery; leaves open prospective studies on optimal management.
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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Amponsah et al. (2026) conducted a review in Unicuspid aortic valve. Unicuspid aortic valve is a rare congenital heart disease presenting in the third to fourth decade of life that requires accurate differentiation from bicuspid aortic valve for proper management.
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