Objective: Patients with Turner syndrome (TS) have an increased risk of aortic dilatation (AD), which is associated with higher mortality. The diagnosis of AD in children is more difficult than that in adults. This study aimed to investigate the application of cardiovascular assessment criteria in diagnosing AD in Chinese children and adolescents with TS.
Methods: In this retrospective study, a comprehensive cardiovascular assessment was performed, according to the 2016 Cincinnati guidelines, in all patients with TS registered from 2017 to 2022 in the Shenzhen Children’s Hospital. In total, 97 girls with TS (1.8-18 years) were included. Patients without structural congenital heart disease were divided into different body surface area groups. Accordingly, Chinese TS population Z-scores (CHTSZ-score) of the ascending aorta diameters were calculated for the AD cases and were compared with the TS-specific Z-score (TSZ-score).
Results: Aortic coarctation (CoA) was present in 12.4% (n = 12) of the patients. Eleven patients had AD (11.3%). The TSZ-score (ranged: -0.94-3.3) for AD diagnosis was significantly lower than the CHTSZ-scores (ranged: -0.76-8.6, P=0.009). The sensitivity to diagnose AD increased to 81.8% when a combination of the TSZ-score, aortic size index (ASI), and the ratio of the ascending to descending aortic diameter (A/D ratio) was applied in the assessment.
Conclusions: The combination of the ASI, TSZ-score, and A/D ratio may increase the sensitivity of the AD diagnosis. A reference for TSZ scores of children and adolescents with TS in China is needed.
Keywords: Turner syndrome, aortic dilatation, ascending aorta diameter, Turner syndrome-specific Z-scores
15 Sep 2022 - 17 Sep 2022