Thunström Sofia, Bech-Hanssen Odd, Krantz Emily, Bryman Inger, Landin-Wilhelmsen Kerstin
Department of Clinical Genetics, Sahlgrenska University Hospital Gothenburg, Sweden.
Department of Internal Medicine and Clinical Nutrition, Institution of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
Int J Cardiol Congenit Heart Dis. 2023 Dec 15;15:100489. doi: 10.1016/j.ijcchd.2023.100489. eCollection 2024 Mar.
Aortic dilation, cardiac malformations and hypertension are known risk factors for aortic dissection in Turner syndrome (TS). In the current guidelines, rapid growth of the aorta has been added as a risk marker. This study aimed to estimate the growth of the ascending aorta over time, to identify risk factors of aortic growth, and to describe aortic complications in TS.
A transthoracic echocardiogram was performed at least twice in 101 women with TS, mean age 28 years, with a mean follow-up of 8.3 ± 3.4 (range 1-17) years. The investigator was blinded to the clinical status. Logistic regression analysis was used to identify risk factors of aortic growth.
The prevalence of ascending aortic dilation (ASI >20 mm/m) was 26 % and the mean ascending aortic diameter was 27.0 ± 4.8 mm at baseline. Significant aortic growth was found at sinus of Valsalva 1.08 (±2.11) mm, sinotubular junction 1.07 (±2.23) mm, and the ascending aorta 2.32 (±2.93) mm, p < 0.001. The mean ascending aortic growth rate was 0.25 (±0.35) mm/year, and higher compared to the general female population, 0.12 (±0.05) mm/year, p < 0.0001. No risk factors for aortic growth (bicuspid aortic valve, coarctatio, hypertension or karyotype) other than body weight could be identified, Odds ratio 1.05 (95 % CI 1.00-1.09), p = 0.029. Eight women had an aortic event of whom all had bicuspid aortic valves.
The growth rate of the ascending aorta in TS was increased compared to the general female population. Congenital cardiovascular malformations were not predictive of aortic growth.
主动脉扩张、心脏畸形和高血压是已知的特纳综合征（TS）患者发生主动脉夹层的危险因素。在当前指南中，主动脉的快速生长已被列为一项风险指标。本研究旨在评估升主动脉随时间的生长情况，确定主动脉生长的危险因素，并描述TS患者的主动脉并发症。
对101例平均年龄28岁的TS女性患者进行了至少两次经胸超声心动图检查，平均随访时间为8.3±3.4（范围1 - 17）年。研究者对临床状况不知情。采用逻辑回归分析确定主动脉生长的危险因素。
升主动脉扩张（主动脉窦指数>20 mm/m）的患病率为26%，基线时升主动脉平均直径为27.0±4.8 mm。在主动脉瓣窦处发现显著的主动脉生长为1.08（±2.11）mm，窦管交界处为1.07（±2.23）mm，升主动脉为2.32（±2.93）mm，p < 0.001。升主动脉平均生长速率为0.25（±0.35）mm/年，高于普通女性人群的0.12（±0.05）mm/年，p < 0.0001。除体重外，未发现其他主动脉生长的危险因素（二叶式主动脉瓣、主动脉缩窄、高血压或核型），比值比为1.05（95%可信区间1.00 - 1.09），p = 0.029。8名女性发生了主动脉事件，她们均患有二叶式主动脉瓣。
与普通女性人群相比，TS患者升主动脉的生长速率有所增加。先天性心血管畸形并非主动脉生长的预测因素。
