Kang Jihee, Kim Young-Wook, Kim Duk-Kyung, Woo Shin-Young, Park Yang-Jin
Division of Vascular Surgery, Department of Surgery, Inha University Hospital, Inha University School of Medicine, Joong-gu, Incheon, Korea.
Division of Vascular Surgery, Department of Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seodaemun-gu, Seoul, Korea.
J Vasc Surg. 2021 Oct;74(4):1163-1171. doi: 10.1016/j.jvs.2021.03.040. Epub 2021 Apr 19.
Marfan syndrome (MFS) affects the cardiovascular system. Aortic root aneurysm is a pathognomonic feature of MFS; however, the abdominal aorta is rarely affected. A consensus on surveillance for the abdominal aorta in patients with MFS has not been established. In the present study, we compared the outcomes after open surgical repair (OSR) of abdominal aortic aneurysms (AAAs) in patients with and without MFS.
We conducted a retrospective, single-center cohort study from 2003 to 2020. We reviewed and compared 28 patients with MFS and 426 patients without MFS who had undergone OSR for AAAs. The baseline characteristics, medical comorbidities, previous cardiovascular surgery, anatomic features of the AAAs, and surgical treatment outcomes were compared between the two groups.
The patients with MFS were younger than those without MFS at the AAA diagnosis (47.2 ± 12.3 vs 70.6 ± 7.9 years; P < .001). The proportion of women was also greater for those with MFS (46.4% vs 15.7%; P < .001). The AAAs were most often located at the infrarenal aorta in both groups. However, thoracoabdominal AAAs were more often found among patients with MFS (10.7% vs 0.9%; P < .012). The proportion of symptomatic patients was lower in the MFS group (3.6% vs 21.6%; P = .022). The maximum median diameter of the AAA at surgery was smaller in the patients with MFS (52 mm vs 58 mm; P = .001). However, concomitant aortic dissection (32.1% vs 3.3%; P < .001) was more prevalent among the patients with MFS. Consequent aneurysmal changes in the iliac artery after AAA repair were more frequent in the patients with MFS (7.1% vs 0%; P = .004). No significant differences were found in 30-day or overall mortality between the patients with and without MFS during a median follow-up period of 71 months (interquartile range, 24.7-121.1 months) and 26.7 months (interquartile range, 7.4-69.5 months), respectively.
The surgical outcomes of OSR for AAAs for patients with MFS were not significantly different from those for patients without MFS in a well-established surveillance program of MFS.
马凡综合征（MFS）会影响心血管系统。主动脉根部瘤是MFS的特征性表现；然而，腹主动脉很少受累。目前尚未就MFS患者腹主动脉的监测达成共识。在本研究中，我们比较了有和没有MFS的患者腹主动脉瘤（AAA）开放手术修复（OSR）后的结果。
我们进行了一项2003年至2020年的回顾性单中心队列研究。我们回顾并比较了28例接受AAA OSR的MFS患者和426例无MFS患者。比较了两组患者的基线特征、合并症、既往心血管手术史、AAA的解剖特征和手术治疗结果。
AAA诊断时，MFS患者比无MFS患者年轻（47.2±12.3岁 vs 70.6±7.9岁；P<.001）。MFS患者中女性比例也更高（46.4% vs 15.7%；P<.001）。两组中AAA最常位于肾下腹主动脉。然而，胸腹主动脉瘤在MFS患者中更常见（10.7% vs 0.9%；P<.012）。MFS组有症状患者的比例较低（3.6% vs 21.6%；P=.022）。MFS患者手术时AAA的最大中位直径较小（52mm vs 58mm；P=.001）。然而，MFS患者中合并主动脉夹层的情况更普遍（32.1% vs 3.3%；P<.001）。AAA修复后髂动脉出现动脉瘤性改变在MFS患者中更常见（7.1% vs 0%；P=.004）。在中位随访期分别为71个月（四分位间距，24.7 - 121.1个月）和26.7个月（四分位间距，7.4 - 69.5个月）时，有和没有MFS的患者在30天或总体死亡率方面没有显著差异。
在完善的MFS监测计划中，MFS患者AAA的OSR手术结果与无MFS患者的结果没有显著差异。
