Division of Cardiology, Department of Medicine, Cardiovascular Imaging Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Am J Cardiol. 2012 Feb 1;109(3):423-7. doi: 10.1016/j.amjcard.2011.09.030. Epub 2011 Nov 29.
Among the cardiovascular manifestations in the Marfan syndrome (MFS), aortic dissection stands out as a major cause of early mortality. The aim of this study was to test the hypothesis that patients with the MFS who experience aortic dissection differ in clinical features and outcomes from those with aortic dissection not related to the MFS. Data from patients diagnosed with aortic dissection from December 1994 to March 2009 at 1 of the major medical centers in Korea were reviewed. The clinical presentations, dissection characteristics, and outcomes of patients with and those without the MFS in a Korean population were compared. Of 445 patients with aortic dissection, 46 (10%) had the MFS. Compared to non-MFS patients, those with the MFS developed aortic dissection at younger ages (33 ± 10 vs 57 ± 13 years, p <0.001) and were less frequently hypertensive (11% vs 73%, p <0.001). During the follow-up period, patients with the MFS more often developed aortic dilatation and expansion of the dissection (39% vs 18%, p = 0.003) and showed a higher rate of reoperation (30% vs 9%, p <0.001). In conclusion, in Korean patients, aortic dissection with the MFS had different characteristics and poorer outcomes than aortic dissection without the MFS. These findings underscore the importance of accurate diagnosis and surveillance of this condition in the MFS.
在马凡综合征（MFS）的心血管表现中，主动脉夹层是导致早期死亡的主要原因之一。本研究旨在验证以下假设：患有 MFS 并发生主动脉夹层的患者在临床特征和结局方面与非 MFS 相关的主动脉夹层患者存在差异。对 1994 年 12 月至 2009 年 3 月在韩国一家主要医疗中心诊断为主动脉夹层的患者的数据进行了回顾。比较了韩国人群中 MFS 患者和非 MFS 患者的临床表现、夹层特征和结局。在 445 例主动脉夹层患者中，有 46 例（10%）患有 MFS。与非 MFS 患者相比，患有 MFS 的患者发生主动脉夹层的年龄更小（33±10 岁 vs 57±13 岁，p<0.001），且高血压发生率更低（11% vs 73%，p<0.001）。在随访期间，患有 MFS 的患者主动脉扩张和夹层扩展的发生率更高（39% vs 18%，p=0.003），再次手术的发生率也更高（30% vs 9%，p<0.001）。总之，在韩国患者中，MFS 合并主动脉夹层的特征和结局与非 MFS 相关的主动脉夹层不同。这些发现强调了在 MFS 中准确诊断和监测该疾病的重要性。
