Turner syndrome (TS) is a chromosomal disorder that affects 1:2000 women. Clinical features of TS include short stature, delayed puberty and congenital cardiac malformations. Turner syndrome patients have an increased prevalence of cardiometabolic risk factors, which predisposes them to complications like coronary artery disease, cerebrovascular-related deaths and aortic dissection. We present, a 35-year-old asymptomatic woman with TS who was referred to our institution because of dilatation of the ascending aorta. Laboratory assessment revealed an increased level of N-terminal pro B-type natriuretic peptide (NT-proBNP; 1510 pg/mL) and anaemia. The chest X-ray showed a widening of the mediastinum (Panel A). Transthoracic echocardiographic study showed giant ascending aortic aneurysm (92 mm) (Panel B) with dissection (see arrow in Panel B). Additionally, severe aortic stenosis (V_max 4.82 m/s) and moderate regurgitation was found. Computer tomography (CT) revealed stenosis of the unicuspid aortic valve (Panel C, see Supplementary material online, VideoS1) and giant ascending and arch aortic aneurysm (98 mm) with dissection reaching the brachiocephalic trunk (Panel D, arrow). Additionally, CT discovered mild coarctation of the descending aorta (16 mm) (see arrow in Panel E), bilateral partial anomalous pulmonary venous return the right upper pulmonary vein (RUPV) returned to the superior vena cave (SVC), and left upper pulmonary vein (LUPV) returned to the innominate vein (PanelsF–H) and the horseshoe kidney (PanelsD and E). The patient underwent successful Bentall operation following a Heart Team discussion.

中文翻译：

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特纳综合征的主动脉无症状解剖提升动脉瘤

特纳综合征（TS）是一种影响1：2000妇女的染色体疾病。TS的临床特征包括身材矮小，青春期延迟和先天性心脏畸形。特纳综合征患者的心脏代谢危险因素患病率上升，这使他们易患冠心病，脑血管相关死亡和主动脉夹层等并发症。我们介绍了一名35岁无症状的TS患者，由于升主动脉扩张而被转诊到我们的机构。实验室评估显示，N末端前B型利尿钠肽（NT-proBNP; 1510 pg / mL）和贫血水平升高。胸部X线片显示纵隔变宽（图A）。经胸超声心动图研究显示巨大的升主动脉瘤（92毫米）（剖开的B板）（请参见B板中的箭头）。此外，还发现了严重的主动脉瓣狭窄（V_最大4.82 m / s）和中度反流。计算机断层扫描（CT）显示单尖瓣主动脉瓣狭窄（面板C，请参见在线补充材料，视频S1）以及巨大的升主动脉和弓状主动脉瘤（98毫米），并且解剖达到臂状脑干（面板D，箭头）。此外，CT发现降主动脉有轻度缩窄（16毫米）（请参阅图E中的箭头）。），双侧局部异常肺静脉返回右上肺静脉（RUPV）返回上腔静脉（SVC），左上肺静脉（LUPV）返回无名静脉（F–H面板）和马蹄肾（面板D和E）。在经过心脏小组讨论后，患者接受了成功的Bentall手术。