Risk profile and operative outcomes in patients with and without Marfan syndrome undergoing thoracoabdominal aortic aneurysm repair

Objectives: To compare operative and long-term outcomes between patients with and without Marfan syndrome undergoing thoracoabdominal aortic aneurysm repair.

Methods: We identified all consecutive patients undergoing thoracoabdominal aortic aneurysm repair between 1997 and 2022. Primary outcome was composite of major adverse events. Secondary outcomes were individual complications and long-term survival. Inverse probability of treatment weighting was performed. Weighted Kaplan-Meier curves were used to estimate long-term survival. Multivariable analysis identified factors associated with major adverse events.

Results: Six hundred eighty-four patients underwent open thoracoabdominal aortic aneurysm repair. Ninety (13.1%) had Marfan syndrome, whereas 594 (86.9%) did not. Marfan patients were younger (46 years [range, 36-56 years] vs 69 years [range, 61-76 years]; P < .001). Extent II or III aneurysms (57 out of 90 [63.3%] vs 211 out of 594 [35.6%]; P < .001) and type I or III chronic dissection (77 out of 90 [85.3%] vs 242 out of 594 [40.8%]; P < .001) were more common. Cardiovascular risk factors were less frequent in Marfan patients. There was no difference in major adverse events between groups (12 out of 90 [13.3%] vs 100 out of 594 [16.8%]; P = .49). Operative mortality was similar between groups (3 out of 90 [3.3%] vs 28 out of 594 [4.7%]; P = .75). Unweighted survival at 10 years was 78.7% vs 46.8% (P = .001). Weighted Kaplan-Meier curves showed no difference in long-term survival (adjusted hazard ratio, 0.79; 95% CI, 0.32-1.99; P = .62; Log-rank P = .12). At multivariable analysis, renal insufficiency (odds ratio, 2.29; 95% CI, 1.43-3.68; P < .01) and urgent/emergency procedure (odds ratio, 2.17; 95% CI, 1.35-3.48; P < .01) were associated with major adverse events, whereas Marfan syndrome was not (odds ratio, 1.56; 95% CI, 0.69-3.49; P = .28).

Conclusions: Open thoracoabdominal aortic aneurysm repair can be performed with similar operative outcomes in patients with and without Marfan syndrome despite differing risk profiles. Operative/perioperative strategies must be tailored to specific needs of each patient to optimize outcomes.