Prevalence of Bicuspid Aortic Valve in Turner Syndrome Patients Receiving Cardiac MRI and CT: A Meta-Analysis

Turner syndrome (TS) is a rare disorder affecting 25–50 in 100000 female newborns. Bicuspid aortic valve (BAV)is assumed to be the most common congenital heart defect (CHD) in TS. In literature, reported BAV prevalence inTS ranges between 14% and 34%. The specific BAV prevalence in TS is still unknown. The aim of this study was togive a more precise estimation of BAV prevalence in TS by conducting a meta-analysis of TS-studies, whichdetected BAV by either cardiac magnetic resonance imaging (MRI) or cardiac computed tomography (CT).We searched PubMed, Cochrane Library, and Web of Science databases to collect observational studies includingthe prevalence of BAV identified by cardiac MRI or cardiac CT in TS patients up to June 4th, 2021. After screeningfor inclusion, data extraction, and quality assessment by two independent reviewers, the meta-analysis was performed with R 4.1.1 software. Results are shown as proportion and weighted mean difference with 95% confi-dence intervals (95% CI). In total, 11 studies involving 1177 patients were included. Pooled data showed thatthe prevalence of BAV in TS patients was 23.7% (95% CI: 21.3% to 26.1%). No high heterogeneity was foundbetween the included studies. The current meta-analysis reveals that BAV can be detected in 23.7% of TS patientsreceiving cardiac MRI or cardiac CT. Therefore, BAV can be considered as the most common CHD in TS. Compared to TTE, cardiac MRI and cardiac CT might represent superior imaging modalities in BAV assessment ofadult TS patients.