LAUSR

OBJECTIVE 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 this study, we aimed to compare the outcomes after open surgical repair (OSR) of abdominal aortic aneurysms (AAAs) in patients with and without MFS. METHODS We conducted a retrospective single-center cohort study between 2003 and 2020. We reviewed and compared 28 patients with MFS and 426 patients without MFS who underwent OSR for AAAs. Baseline characteristics, medical comorbidities, history of cardiovascular surgery, anatomic features of AAA, and surgical treatment outcomes were compared between the two groups. RESULTS Patients with MFS were younger than those without MFS at the time of AAA diagnosis (47.2±12.3 vs. 70.6±7.9 years, P < .001). The proportion of females was also higher in MFS (46.4% vs. 15.7%, P <.001). AAA was most commonly located at the infrarenal aorta in both groups; however, thoracoabdominal AAA was more commonly found among patients with MFS (10.7% vs. 0.9%, P <.012). The proportion of symptomatic patients was lower in MFS (3.6% vs. 21.6%, P = .022). The maximum median diameter of the aneurysm at the time of operation was smaller in 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 patients with MFS. Consequent aneurysmal change of the iliac artery after AAA operation was more frequent in patients with MFS (7.1% vs. 0%, P=.004). There was no significant difference in 30-day or overall mortality between patients with and without MFS during a median follow-up period of 71 (interquartile range: 24.7-121.1) and 26.7 (interquartile range: 7.4-69.5) months, respectively. CONCLUSIONS The surgical outcomes of OSR for AAAs in patients with MFS were not significantly different from patients without MFS in a well-established surveillance program of MFS.