背景:特纳综合征(TS)患者发生主动脉扩张(AD)的风险增加,但与成人相比,儿童AD的诊断具有更大的复杂性。本研究旨在探讨各种AD评估指标在中国儿童青少年TS中的应用。
方法:本研究纳入2017-2022年深圳市儿童医院收治的TS患者。心血管病变由经验丰富的放射科医生诊断。没有结构性心脏病的患者被分为不同的体表面积组,然后计算升主动脉的中国TS人群Z评分(CHTSZ评分),并与其他指标如主动脉大小指数(ASI)进行比较,升主动脉与降主动脉直径之比(A/D比),和TSZ分数(Quezada\的方法)。
结果:共纳入115例TS患者,平均年龄为10.0±3.7岁。三种最严重的心血管并发症(AD)的发生率为9.6%,10.4%(主动脉缩窄,CoA),和7.0%(二叶主动脉瓣,BAV),分别。≥10岁的TS患者发生AD的比例高于<10岁的患者(16.6%vs.1.8%,P=0.009),另外出现AD的CoA或BAV患者的比例高于没有这些情况的患者(31.6%vs.5.2%,P<0.001)。ASI,A/D比,TSZ-得分,11例AD患者的CHTSZ评分分别为2.27±0.40cm/m2、1.90±0.37、1.28±1.08和3.07±2.20。在AD患者中,只有3例TSZ评分≥2,2例TSZ评分≥1.然而,根据使用CHTSZ分数的评估,6例患者评分≥2,5例患者评分≥1。相比之下,与CHTSZ评分相比,TSZ评分总体上低估了中国TS患儿的主动脉Z评分.
结论:ASI和A/D比率对TS儿童的适用性值得怀疑,种族差异会影响中国人群TSZ得分的评估。因此,建立专门为中国儿童和青少年量身定制的CHTSZ评分至关重要。
BACKGROUND: Patients with Turner syndrome (TS) face an increased risk of developing aortic dilatation (AD), but diagnosing AD in children presents greater complexity compared to adults. This study aimed to investigate the application of various assessment indicators of AD in Chinese children and adolescents with TS.
METHODS: This study included TS patients admitted to Shenzhen Children\'s Hospital from 2017 to 2022. Cardiovascular lesions were diagnosed by experienced radiologists. Patients without structural heart disease were divided into different body surface area groups, then the Chinese TS population Z-score (CHTSZ-score) of the ascending aorta was calculated and compared with other indicators such as aortic size index (ASI), ratio of the ascending to descending aortic diameter (A/D ratio), and TSZ-score (Quezada\'s method).
RESULTS: A total of 115 TS patients were included, with an average age of 10.0 ± 3.7 years. The incidences of the three most serious cardiovascular complications were 9.6% (AD), 10.4% (coarctation of the aorta, CoA), and 7.0% (bicuspid aortic valve, BAV), respectively. The proportion of developing AD in TS patients aged ≥ 10 years was higher than that in those < 10 years old (16.6% vs. 1.8%, P = 0.009), and the proportion of patients with CoA or BAV who additionally exhibited AD was higher than those without these conditions (31.6% vs. 5.2%, P < 0.001). The ASI, A/D ratio, TSZ-score, and CHTSZ-score of the 11 patients with AD were 2.27 ± 0.40 cm/m2, 1.90 ± 0.37, 1.28 ± 1.08, and 3.07 ± 2.20, respectively. Among the AD patients, only 3 cases had a TSZ-score ≥ 2, and 2 cases had a TSZ-score ≥ 1. However, based on the assessment using the CHTSZ-score, 6 patients scored ≥ 2, and 5 patients scored ≥ 1. In contrast, the TSZ-score generally underestimated the aortic Z-scores in Chinese children with TS compared to the CHTSZ-score.
CONCLUSIONS: The applicability of ASI and A/D ratio to children with TS is questionable, and racial differences can affect the assessment of TSZ-score in the Chinese population. Therefore, establishing the CHTSZ-score specifically tailored for Chinese children and adolescents is of paramount importance.