Natural history of aortic root aneurysms in Marfan syndrome
Natural history of aortic root aneurysms in Marfan syndrome
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DOI：
10.21037/acs.2017.11.10
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发表时间：
2017-11-01
影响因子：
3.1
通讯作者：
Elefteriades, John A.
中科院分区：
文献类型：
--
作者：
Saeyeldin, Ayman;Zafar, Mohammad A.;Elefteriades, John A.
背景：心血管并发症在马凡综合征(MFS)患者寿命缩短中占很大比例，其中主动脉夹层是最可怕的并发症。在MFS患者中，主动脉根部最初扩张，由于其重要的血流动力学作用，这可能导致主动脉瓣反流，并构成主动脉夹层的巨大风险。本研究旨在评估MFS患者的主动脉根部动脉瘤的自然病史，重点是生长率以及根部直径与发生主动脉并发症的风险的相关性。方法：对78例MFS合并主动脉根部扩张的患者进行回顾性分析，并对其主动脉根部直径进行系列分析。结果：主动脉根部平均年生长率为0.26±0.05 cm/年(0.13~0.35 cm)。较大的动脉瘤生长更快，高达0.46厘米/年。主动脉夹层的平均发病年龄为36±4岁。用Logistic回归模型分别计算3.5、4、4.5、5、5.5和6 cm的不良事件(破裂、夹层和死亡)的年发生率。直径5.5~6 cm的患者发生并发症的风险急剧增加23%。结论：与非MFS患者相比，MFS患者的主动脉根部动脉瘤的扩张速度更快，主动脉根部直径对每年发生主动脉并发症的风险有显著影响。
Background: Cardiovascular complications account for a significant proportion of the shortened lifespan of Marfan syndrome (MFS) patients, with aortic dissection being the most dreadful complication. The aortic root dilates initially in MFS patients, and given its important hemodynamic role, this can lead to aortic regurgitation and poses a substantial risk of aortic dissection. This study seeks to evaluate the natural history of aortic root aneurysms in MFS patients, with a focus on growth rates and correlation of root diameter with the risk of developing aortic complications.Methods: Seventy-eight patients confirmed to have MFS and aortic root dilatation were retrospectively reviewed, and their aortic root diameters serially analyzed. Annual growth rate estimates and yearly rates of adverse events were computed and correlated with aortic diameter.Results: The mean annual growth rate of the aortic root was estimated to be 0.26 +/- 0.05 cm/year (range 0.13 to 0.35 cm). Larger aneurysms grew faster, reaching up to 0.46 cm/year for aneurysms >6 cm. Mean age at onset of aortic dissection was 36 +/- 4 years. Annual rates of adverse events (rupture, dissection and death) were obtained using a logistic regression model at sizes 3.5, 4, 4.5, 5, 5.5 and 6 cm. A sharp increase of 23% in the probability of the risk of complications at diameters 5.5 to 6 cm was recognized.Conclusion: Aortic root aneurysms in MFS patients tend to have a faster expansion rate compared to non-MFS individuals, with aortic root diameter having a significant impact on the yearly risk of developing aortic complications.