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

Read at the 103rd Annual Meeting of The American Association for Thoracic Surgery, Los Angeles, California, May 6-9, 2023.
https://doi.org/10.1016/j.jtcvs.2023.03.029Get rights and content

Abstract

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.

Section snippets

Methods

The departmental aortic surgery database was reviewed to identify all consecutive patients who underwent first-time open TAAA repair between May 1997 and April 2022. This prospectively maintained database was queried for pre-, intra- and postoperative details. The database is prospectively maintained by chart review. A review of the collected patients' health information was conducted and missing or outdated data were retrieved or updated through review of medical records. This study was

Results

A total of 684 patients underwent open TAAA repair during the study period. Of these, 90 (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), had lower ejection fraction (P = .03), had more frequently undergone previous heart surgery (85.6% vs 52.9%; P < .001), including proximal aortic graft implantation (43.3% vs 14.1%; P < .001) and aortic valve replacement (35.6% vs 4.0%; P < .001).

Discussion

Outcomes of open surgical repair of TAAA have significantly improved during the past few decades due to the development and improvement in preoperative risk assessment, intraoperative techniques, and postoperative intensive care. This includes aggressive intraoperative approaches like neuroprotective strategies, intercostal reimplantation,19 visceral and renal perfusion,20,21 left heart bypass,22 and hypothermic circulatory arrest.4 Despite all of these improvements in managing patients with

Conclusions

Open repair of TAAA in patients with Marfan syndrome can be performed with similar operative outcomes to patients without Marfan syndrome despite the association with higher risk aneurysm features in Marfan patients and cardiovascular risk factors in patients without Marfan syndrome. Due to the vastly different risk profiles, operative and perioperative strategies must differ and surgeons need to tailor the operation to the specific needs of each patient to optimize outcomes. By mitigating the

References (36)

Drs Lau and Soletti contributed equally to this article.

IRB#: 1607017424 (approved January 9, 2022), need for informed consent waived.

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