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Abstract：
OBJECTIVE: To determine the prevalence of proximal aortic abnormalities in patients with Ehlers-Danlos syndrome (EDS).
METHODS: In a prospective cohort study, aortic measurements by two-dimensional echocardiography were performed on consecutive EDS patients.
RESULTS: Twenty-eight percent (20 of 71) had aortic root dilation (ARD) (> +2 SD above population based norms). Fourteen of 42 individuals with the classical form of EDS (types I/II) and 6 of 29 individuals with the hypermobile form (type III) had ARD, with no gender differences.
CONCLUSIONS: ARD is a common finding in EDS. Longitudinal studies are indicated to determine progression of ARD and its clinical significance.
METHODS: In a prospective cohort study, aortic measurements by two-dimensional echocardiography were performed on consecutive EDS patients.
RESULTS: Twenty-eight percent (20 of 71) had aortic root dilation (ARD) (> +2 SD above population based norms). Fourteen of 42 individuals with the classical form of EDS (types I/II) and 6 of 29 individuals with the hypermobile form (type III) had ARD, with no gender differences.
CONCLUSIONS: ARD is a common finding in EDS. Longitudinal studies are indicated to determine progression of ARD and its clinical significance.
摘要：
目的：确定Ehlers-Danlos综合征（EDS）患者近端主动脉异常的发生率。
方法：在一项前瞻性队列研究中，通过二维超声心动图对连续EDS患者进行主动脉测量。
结果：28％（71个中的20个）的主动脉根部扩张（ARD）（比基于人群的标准高出2SD）。具有EDS经典形式（I/II型）的42个人中有14个人和具有高移动形式（III型）的29个人中有6个人患有ARD，没有性别差异。
结论：ARD是EDS中的一个常见发现。纵向研究表明确定ARD的进展及其临床意义。
方法：在一项前瞻性队列研究中，通过二维超声心动图对连续EDS患者进行主动脉测量。
结果：28％（71个中的20个）的主动脉根部扩张（ARD）（比基于人群的标准高出2SD）。具有EDS经典形式（I/II型）的42个人中有14个人和具有高移动形式（III型）的29个人中有6个人患有ARD，没有性别差异。
结论：ARD是EDS中的一个常见发现。纵向研究表明确定ARD的进展及其临床意义。
