Overview
Unicuspid aortic valve (UAV) is a rare congenital valvular anomaly that can be misdiagnosed as the more common bicuspid aortic valve (BAV) 1. It is characterized by a single commissure 1.Diagnosis
Diagnosis is typically performed with transthoracic echocardiography (TTE) and confirmed with transesophageal echocardiography (TEE), potentially including 3D-TEE 1.
Associated conditions include aortic coarctation (3.8-12%), ventricular septal defect (3%), and Turner syndrome (3%) 1.
Dilated ascending aorta is frequently observed, present in 44% of retrospective studies and 35% of case reports 1.Management
The most common surgical intervention is aortic valve replacement (AVR) 1.
Concomitant ascending aorta replacement or repair is reported in 38% of retrospective studies and 27% of case reports 1.Special Populations
Studies included patients aged 14 years and older 1.
UAV is more common in males (76-79%) 1.
The mean age at diagnosis in adults is 36 years 1.Key Recommendations
Unicuspid aortic valve should be considered a distinct entity separate from bicuspid aortic valve 1. (Evidence: Moderate)
Transthoracic echocardiography is a primary diagnostic tool, with transesophageal echocardiography used for confirmation 1. (Evidence: Moderate)
Aortic valve replacement is a common surgical approach for symptomatic UAV 1. (Evidence: Moderate)
Consider surgical repair or replacement of the ascending aorta in patients with UAV and aortic dilation 1. (Evidence: Moderate)References
1 Carbone A, Ferrara F, Bottino R, Russo V, Rega S, Limongelli G et al.. Current evidence of unicuspid aortic valve in young adults: A systematic review and metanalysis. Current problems in cardiology 2025. link