Abstract：
Although tissue stiffness is known to play an important role in aortic dilatation, the current guidelines for offering preventative surgery in patients with Marfan syndrome rely solely on the aortic diameter. In this systematic review and meta-analysis, we analyze and compare literature on in vivo aortic stiffness measures in Marfan patients. Our aim is to assess the potential of these measurements as early indicators of aortic dilatation. Following the PRISMA guidelines, we collected literature on diameter and three in vivo stiffness measures: Pulse wave velocity (PWV), β -stiffness index (SI) and distensibility, at five different aortic locations in patients with Marfan syndrome. Results were reviewed and compared against each other. For meta-analysis, an augmented dataset was created by combining data from the literature. Regression with respect to age and statistical comparisons were performed. Thirty articles reporting data from 1925 patients with Marfan and 836 patients without Marfan were reviewed. PWV was found to be higher in Marfan, but only in dilated aortas. Distensibility was found to be lower even in non-dilated aortas, and its decrease was associated with higher chances of developing aortic dilatation. β -SI was higher in Marfan patients and was positively correlated with the rate of aortic dilatation, emphasizing its role as a valuable indicator. In our meta-analysis, all stiffness measures showed a significant variation with age. Distensibility and β -stiffness index were different in Marfan patients at all locations, and the difference was more pronounced after accounting for age-related variation. From the literature, β -SI and distensibility emerge as the best predictors of future aortic dilatation. Our meta-analysis quantifies age-related changes in aortic stiffness and highlights the importance of accounting for age in comparing these measurements. Missing diameter values in the literature limited our analysis. Further investigation of criteria combining stiffness and diameter is recommended to better assist clinical decisions for prophylactic surgery.
摘要：
尽管已知组织硬度在主动脉扩张中起重要作用，目前为马凡氏综合征患者提供预防性手术的指南仅依赖于主动脉直径.在这篇系统综述和荟萃分析中，我们分析并比较了有关马凡氏患者体内主动脉僵硬度测量的文献。我们的目的是评估这些测量作为主动脉扩张的早期指标的潜力。按照PRISMA准则，我们收集了有关直径和三种体内刚度测量的文献：脉搏波速度（PWV），β-刚度指数(SI)和膨胀性，马凡氏综合征患者的五个不同主动脉位置。对结果进行审查并相互比较。对于荟萃分析，通过结合文献中的数据创建了一个增强数据集.进行年龄回归和统计学比较。回顾了30篇报告1925例马凡族患者和836例无马凡族患者的数据。发现Marfan的PWV更高，但仅限于扩张的主动脉.发现即使在未扩张的主动脉中，扩张性也较低，其减少与主动脉扩张的机会增加有关。马凡尔患者的β-SI较高，与主动脉扩张率呈正相关，强调其作为有价值指标的作用。在我们的荟萃分析中，所有刚度测量均显示出随年龄的显著变化.Marfan患者在所有位置的扩张性和β-刚度指数均不同。在考虑与年龄相关的变化后，差异更为明显。从文学,β-SI和扩张性成为未来主动脉扩张的最佳预测因子。我们的荟萃分析量化了与年龄相关的主动脉僵硬度变化，并强调了在比较这些测量结果时考虑年龄的重要性。文献中缺少的直径值限制了我们的分析。建议进一步研究结合刚度和直径的标准，以更好地协助预防性手术的临床决策。
