Unicuspid aortic valve (UAV) is a rare congenital valvular anomaly, often misdiagnosed as the more prevalent bicuspid aortic valve (BAV). The aim of this study was to explore demographic, clinical characteristics, diagnosis, surgical options, short and long term outcomes of young adults with UAV. A systematic review and meta-analysis of literature were conducted for studies (1971-2024) including patients (≥ 14 years old) with diagnosis of UAV. Among the 2953 studies retrieved, 67 case reports, 6 case series (n = 130) and 13 retrospective studies (n = 918), were included in the analysis. Data from retrospective studies were aggregated using a random effects model for estimating the pooled risk ratio and mean difference. UAV is mostly unicommissural in adults (mean age 36 years old at diagnosis, 76-79 % males). The most common conditions associated in UAV patients were aortic coarctation (from 3.8 to 12 %), ventricular septal defect (3 %) and Turner syndrome (3 %). In general, the diagnosis was performed with TTE and confirmed with TEE (+/- 3D-TEE). The most common types of surgery were AVR. Dilated ascending aorta was described in 44 % and 35 % of retrospective studies and case reports, respectively. Concomitant ascending aorta replacement/repair was reported 38 % and 27 % of retrospective studies and case reports, respectively. Overall survival was reported in 3 studies, ranging from 95 to 98 % at 10 years. UAV should be considered a separate entity from BAV. Further investigations with regards to the possibility of a familial incidence, associated histopathological changes in the aorta, and ideal follow up and intervention are needed.
Keywords: Aortic dilatation; Aortic regurgitation; Aortic stenosis; Bicuspid aortic valve; Unicuspid aortic valve.
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