Meccanici Frederike, Schotte Mathilda H, Snoeren Miranda, Bons Lidia R, van den Hoven Allard T, Kardys Isabella, Budde Ricardo P J, van den Bosch Annemien E, Duijnhouwer Anthonie L, Roos-Hesselink Jolien W
Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands.
Radiology and Nuclear Medicine, Radboudumc, Nijmegen, The Netherlands.
Heart. 2022 Dec 22;109(2):102-110. doi: 10.1136/heartjnl-2022-320922.
Women with Turner syndrome (TS) are at increased risk of aortic dissection, which is a life-threatening event associated with aortic dilation. Knowledge on the development of aortic dilation over time remains limited. This study aims to describe the prevalence of aortic dilation, to find associated factors and to study aortic growth in women with TS.
In this prospective multicentre cohort study, consecutive adult women with genetically proven TS included between 2014 and 2016 underwent ECG-triggered multiphase CT angiography at baseline and after 3 years. Aortic diameters were measured at seven levels of the thoracic aorta using double oblique reconstruction and indexed for body surface area. Ascending aortic dilation was defined as an aortic size index >20 mm/m. Aorta-related and cardiovascular events were collected. Statistical analysis included linear and logistic regression and linear mixed effects models.
The cohort consisted of 89 women with a median age of 34 years (IQR: 24-44). Ascending aortic dilation was found in 38.2% at baseline. At baseline, age (OR: 1.08 (95% CI 1.03 to 1.13), p<0.001), presence of bicuspid aortic valve (BAV) (OR: 7.09 (95% CI 2.22 to 25.9), p=0.002) and systolic blood pressure (OR: 1.06 (95% CI 1.02 to 1.11), p=0.004) were independently associated with ascending aortic dilation. During a median follow-up of 3.0 (2.4-3.6) years (n=77), significant aortic growth was found only at the sinotubular junction (0.20±1.92 mm, p=0.021). No aortic dissection occurred, one patient underwent aortic surgery and one woman died.
In women with TS, ascending aortic dilation is common and associated with age, BAV and systolic blood pressure. Aortic diameters were stable during a 3-year follow-up, apart from a significant yet not clinically relevant increase at the sinotubular junction, which may suggest a more benign course of progression than previously reported.
特纳综合征（TS）女性发生主动脉夹层的风险增加，主动脉夹层是一种与主动脉扩张相关的危及生命的事件。关于主动脉扩张随时间发展的认识仍然有限。本研究旨在描述主动脉扩张的患病率，找出相关因素，并研究TS女性的主动脉生长情况。
在这项前瞻性多中心队列研究中，2014年至2016年间纳入的经基因证实的成年TS女性在基线时和3年后接受了心电图触发的多期CT血管造影。使用双斜重建在胸主动脉的七个水平测量主动脉直径，并根据体表面积进行指数化。升主动脉扩张定义为主动脉大小指数>20 mm/m²。收集与主动脉相关和心血管事件。统计分析包括线性和逻辑回归以及线性混合效应模型。
该队列由89名女性组成，中位年龄为34岁（四分位间距：24 - 44岁）。基线时发现38.2%的患者有升主动脉扩张。在基线时，年龄（比值比：1.08（95%置信区间1.03至1.13），p<0.001）、二叶式主动脉瓣（BAV）的存在（比值比：7.09（95%置信区间2.22至25.9），p = 0.002）和收缩压（比值比：1.06（95%置信区间1.02至1.11），p = 0.004）与升主动脉扩张独立相关。在中位随访3.0（2.4 - 3.6）年（n = 77）期间，仅在窦管交界处发现显著的主动脉生长（0.20±1.92 mm，p = 0.021）。未发生主动脉夹层，1例患者接受了主动脉手术且1名女性死亡。
在TS女性中，升主动脉扩张很常见，且与年龄、BAV和收缩压相关。在3年随访期间，除窦管交界处有显著但无临床意义的增加外，主动脉直径稳定，这可能表明其进展过程比先前报道的更为良性。
