Franken Romy, El Morabit Abdelali, de Waard Vivian, Timmermans Janneke, Scholte Arthur J, van den Berg Maarten P, Marquering Henk, Planken Nils R N, Zwinderman Aeilko H, Mulder Barbara J M, Groenink Maarten
Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of the Netherlands, The Netherlands.
Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands.
Int J Cardiol. 2015 Sep 1;194:7-12. doi: 10.1016/j.ijcard.2015.05.072. Epub 2015 May 15.
Patients with Marfan syndrome (MFS) have a highly variable occurrence of aortic complications. Aortic tortuosity is often present in MFS and may help to identify patients at risk for aortic complications.
3D-visualization of the total aorta by MR imaging was performed in 211 adult MFS patients (28% with prior aortic root replacement) and 20 controls. A method to assess aortic tortuosity (aortic tortuosity index: ATI) was developed and reproducibility was tested. The relation between ATI and age, and body size and aortic dimensions at baseline was investigated. Relations between ATI at baseline and the occurrence of a clinical endpoint (aortic dissection, and/or aortic surgery) and aortic dilatation rate during 3 years of follow-up were investigated.
ATI intra- and interobserver agreements were excellent (ICC: 0.968 and 0.955, respectively). Mean ATI was higher in 28 age-matched MFS patients than in the controls (1.92 ± 0.2 vs. 1.82 ± 0.1, p=0.048). In the total MFS cohort, mean ATI was 1.87 ± 0.20, and correlated with age (r=0.281, p<0.001), aortic root diameter (r=0.223, p=0.006), and aortic volume expansion rate (r=0.177, p=0.026). After 49.3 ± 8.8 months follow-up, 33 patients met the combined clinical endpoint (7 dissections) with a significantly higher ATI at baseline than patients without endpoint (1.98 ± 0.2 vs. 1.86 ± 0.2, p=0.002). Patients with an ATI>1.95 had a 12.8 times higher probability of meeting the combined endpoint (log rank-test, p<0.001) and a 12.1 times higher probability of developing an aortic dissection (log rank-test, p=0.003) compared to patients with an ATI<1.95.
Increased ATI is associated with a more severe aortic phenotype in MFS patients.
马凡综合征（MFS）患者主动脉并发症的发生率差异很大。主动脉迂曲在MFS患者中很常见，可能有助于识别有主动脉并发症风险的患者。
对211例成年MFS患者（28%曾行主动脉根部置换术）和20例对照者进行磁共振成像对整个主动脉的三维可视化检查。开发了一种评估主动脉迂曲的方法（主动脉迂曲指数：ATI）并测试了其可重复性。研究了ATI与年龄、体型以及基线时主动脉尺寸之间的关系。研究了基线时的ATI与临床终点事件（主动脉夹层和/或主动脉手术）的发生以及随访3年期间主动脉扩张率之间的关系。
观察者内和观察者间ATI的一致性都很好（组内相关系数分别为0.968和0.955）。28例年龄匹配的MFS患者的平均ATI高于对照组（1.92±0.2对1.82±0.1，p=0.048）。在整个MFS队列中，平均ATI为1.87±0.20，与年龄（r=0.281，p<0.001）、主动脉根部直径（r=0.223，p=0.006）和主动脉容积扩张率（r=0.177，p=0.026）相关。经过49.3±8.8个月的随访，33例患者达到了联合临床终点（7例夹层），其基线时的ATI显著高于未达到终点的患者（1.98±0.2对1.86±0.2，p=0.002）。与ATI<1.95的患者相比，ATI>1.95的患者达到联合终点的概率高12.8倍（对数秩检验，p<0.001），发生主动脉夹层的概率高12.1倍（对数秩检验，p=0.003）。
MFS患者中ATI升高与更严重的主动脉表型相关。
