摘要
目的：此研究的主要目的是描述马方综合征（MFS）患者发生病理性主动脉根部扩张时的年龄。方法和结果：共对160例在地区心脏中心接受治疗的MFS患者进行了回顾。通过将最大的主动脉窦测量值与文献中的对照资料进行比较来诊断升主动脉扩张。采用Kaplan-Meier生存曲线估计扩张发生时的年龄。所有患者就诊时的平均年龄为15．5岁（极差1．5～40岁）。有95％的患者出现了骨骼畸形。18％的患者累及了眼睛。在160例患者中，有115例发生主动脉根部畸形，其中78例（68％）在19岁之前就发生了主动脉根部扩张。
Aims： The main aim of this study was to describe the age at which pathological aortic root dilation occurs in patients with Marfan＇s syndrome（MFS） . Methods and results： A total of 160 patients with MFS attending a regional cardiac centre were reviewed retrospectively. Dilation of the ascending aorta was diagnosed by comparing the maximum aortic sinus measurement with control data from the literature. We employed a Kaplan-Meier survival curve to estimate the age at which dilatation occurs. The mean age of the total group at presentation was 15.5 years（range 1.5 - 40 years） . Skeletal abnormalities were present in 95%. Eye involvement was found in 18%. In the 115/160 patients with an abnormal aortic root, 78/115（68% ） developed aortic root dilatation before 19 years of age.
