title: Comparison of aortic dissection in korean patients with versus without the marfan syndrome.
abstract: 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.
authors: Division of Cardiology, Department of Medicine, Cardiovascular Imaging Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors: Eun Kyoung
authors: EK
authors: Kim
authors: E Ryoung
authors: ER
authors: Choi
authors: Sung Min
authors: SM
authors: Ko
authors: Shin Yi
authors: SY
authors: Jang
authors: Seung Hyuk
authors: SH
authors: Choi
authors: Chang-Seok
authors: CS
authors: Ki
authors: Yeon Hyeon
authors: YH
authors: Choe
authors: Wook Sung
authors: WS
authors: Kim
authors: Kiick
authors: K
authors: Sung
authors: Jae K
authors: JK
authors: Oh
authors: Duk-Kyung
authors: DK
authors: Kim
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*; D000792:Angiography; D017545:Aortic Aneurysm, Thoracic / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*; D002423:Cause of Death / Q000639:trends; D002908:Chronic Disease; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D008875:Middle Aged; D011379:Prognosis; D056910:Republic of Korea / Q000453:epidemiology; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D014057:Tomography, X-Ray Computed