Objectives Patients with Marfan syndrome (MFS) are prone to develop aortic aneurysms due to fragmentation of elastic fibres, resulting in reduced distensibility of the aorta. Reduced distensibility was previously shown to predict progressive descending aorta dilatation. Here, we investigated longitudinal changes in distensibility, as a potential predictor of aortic events. Methods This retrospective study included all patients with MFS with at least four cardiac magnetic resonance examinations performed between 1996 and 2012. Aortic distensibility was assessed, in the ascending (level 1), proximal descending (level 2) and distal descending (level 3) aorta. Changes in distensibility were studied using linear mixed-effects regression models. Results In total, 35 patients with MFS (age at inclusion 28 (IQR 23–32) years, 54% men) were included. Mean aortic distensibility was already low (between 2.9×10–3/mm Hg/year and 6.4×10–3/mm Hg/year) at all levels at baseline, and significantly decreased over time at levels 2 and 3 (respectively, p=0.012 and p=0.002). The rate of distensibility loss per year (×10-3/mm Hg/year) was 0.01, 0.03 and 0.06×10–3/mm Hg at levels 1, 2 and 3, respectively. At inclusion, men exhibited very low distensibility, whereas women showed moderately reduced distensibility, gradually decreasing with age. Aortic dilatation rate at level 2 was associated with reduced aortic distensibility. However, we could not demonstrate a direct correlation between distensibility and clinical events during a follow-up of 22 years. Conclusion Patients with MFS display reduced aortic distensibility already at an early age, inversely relating to aortic dilatation rate. However, in this selected patient group, distensibility seems less suitable as an individual predictor of aortic events. Data are available upon reasonable request. The data underlying this article will be shared on reasonable request to the corresponding author.

中文翻译：

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马凡综合征的主动脉扩张：主动脉事件的潜在预测因素？

目的 马凡综合征 (MFS) 患者由于弹性纤维的碎裂而容易发生主动脉瘤，从而导致主动脉的扩张性降低。先前显示降低的扩张性可预测进行性降主动脉扩张。在这里，我们调查了可扩张性的纵向变化，作为主动脉事件的潜在预测因子。方法 这项回顾性研究包括在 1996 年至 2012 年期间进行了至少四次心脏磁共振检查的所有 MFS 患者。评估了升主动脉（1 级）、近端降主动脉（2 级）和远端降主动脉（3 级）的主动脉扩张性. 使用线性混合效应回归模型研究可扩张性的变化。结果 共纳入 35 名 MFS 患者（纳入年龄 28（IQR 23-32）岁，54% 为男性）。基线时所有水平的平均主动脉扩张性已经很低（在 2.9×10-3/mm Hg/年和 6.4×10-3/mm Hg/年之间），并且随着时间的推移在 2 级和 3 级显着下降（分别为 p =0.012 和 p = 0.002)。在 1、2 和 3 级时，每年的膨胀性损失率（×10-3/mm Hg/年）分别为 0.01、0.03 和 0.06×10-3/mm Hg。在纳入时，男性表现出非常低的扩张性，而女性表现出适度降低的扩张性，随着年龄的增长逐渐下降。2 级的主动脉扩张率与主动脉扩张性降低有关。然而，在 22 年的随访中，我们无法证明扩张性与临床事件之间存在直接相关性。结论 MFS 患者在很小的时候就表现出主动脉扩张性降低，与主动脉扩张率成反比。然而，在这个选定的患者组中，扩张性似乎不太适合作为主动脉事件的个体预测因子。可根据合理要求提供数据。本文所依据的数据将根据合理要求分享给通讯作者。