Devos Daniel G H, De Groote Katya, Babin Danilo, Demulier Laurent, Taeymans Yves, Westenberg Jos J, Van Bortel Luc, Segers Patrick, Achten Eric, De Schepper Jean, Rietzschel Ernst
Department of Radiology, MRI (-1K12), Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
Pediatric Cardiology, Department of Pediatrics and Turner Clinic, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
J Cardiovasc Magn Reson. 2017 Feb 13;19(1):27. doi: 10.1186/s12968-017-0331-0.
To study segmental structural and functional aortic properties in Turner syndrome (TS) patients. Aortic abnormalities contribute to increased morbidity and mortality of women with Turner syndrome. Cardiovascular magnetic resonance (CMR) allows segmental study of aortic elastic properties.
We performed Pulse Wave Velocity (PWV) and distensibility measurements using CMR of the thoracic and abdominal aorta in 55 TS-patients, aged 13-59y, and in a control population (n = 38;12-58y). We investigated the contribution of TS on aortic stiffness in our entire cohort, in bicuspid (BAV) versus tricuspid (TAV) aortic valve-morphology subgroups, and in the younger and older subgroups.
Differences in aortic properties were only seen at the most proximal aortic level. BAV Turner patients had significantly higher PWV, compared to TAV Turner (p = 0.014), who in turn had significantly higher PWV compared to controls (p = 0.010). BAV Turner patients had significantly larger ascending aortic (AA) luminal area and lower AA distensibility compared to both controls (all p < 0.01) and TAV Turner patients. TAV Turner had similar AA luminal areas and AA distensibility compared to Controls. Functional changes are present in younger and older Turner subjects, whereas ascending aortic dilation is prominent in older Turner patients. Clinically relevant dilatation (TAV and BAV) was associated with reduced distensibility.
Aortic stiffening and dilation in TS affects the proximal aorta, and is more pronounced, although not exclusively, in BAV TS patients. Functional abnormalities are present at an early age, suggesting an aortic wall disease inherent to the TS. Whether this increased stiffness at young age can predict later dilatation needs to be studied longitudinally.
研究特纳综合征（TS）患者主动脉节段的结构和功能特性。主动脉异常会导致特纳综合征女性的发病率和死亡率增加。心血管磁共振成像（CMR）可对主动脉弹性特性进行节段性研究。
我们使用CMR对55例年龄在13 - 59岁的TS患者以及38例年龄在12 - 58岁的对照人群的胸主动脉和腹主动脉进行脉搏波速度（PWV）和扩张性测量。我们在整个队列、二叶式（BAV）与三叶式（TAV）主动脉瓣形态亚组以及年轻和老年亚组中研究了TS对主动脉僵硬度的影响。
仅在主动脉最近端水平观察到主动脉特性的差异。与TAV特纳患者相比，BAV特纳患者的PWV显著更高（p = 0.014），而TAV特纳患者的PWV又显著高于对照组（p = 0.010）。与对照组（所有p < 0.01）和TAV特纳患者相比，BAV特纳患者的升主动脉（AA）管腔面积显著更大，AA扩张性更低。与对照组相比，TAV特纳患者的AA管腔面积和AA扩张性相似。年轻和老年特纳受试者均存在功能变化，而升主动脉扩张在老年特纳患者中更为突出。临床相关的扩张（TAV和BAV）与扩张性降低有关。
TS患者的主动脉硬化和扩张影响近端主动脉，在BAV TS患者中更为明显，尽管并非唯一。功能异常在早年就已出现，提示TS存在主动脉壁疾病。年轻时这种增加的僵硬度是否能预测后期扩张需要进行纵向研究。
