Comparative analysis of long-term outcomes in thoracoabdominal aortic aneurysm repair between Marfan syndrome patients and non-Marfan syndrome patients.

BACKGROUND

A consensus on the management of thoracoabdominal aortic aneurysm (TAAA) in patients with Marfan syndrome (MFS) has not yet been established. This study aimed to compare the long-term outcomes after open TAAA repair in patients with and without MFS.

METHODS

This retrospective study examined 230 consecutive patients who underwent TAAA repair between 2012 and 2022, including of 69 MFS patients and 161 non-MFS patients. The primary endpoint was long-term mortality. The secondary endpoint was a composite of early adverse events, including early mortality, permanent stroke, permanent paraplegia, permanent renal failure, and reoperation. Univariable and multivariable logistic regression analyses were used to assess the impact of MFS on early composite adverse events, and univariable and multivariable Cox proportional hazards models were constructed to evaluate the association between MFS and overall mortality.

RESULTS

Compared with non-MFS patients, MFS patients were younger (mean, 31.9 ± 8.5 years vs 44.8 ± 12.3 years; P < .001), had less comorbid coronary artery disease (0 vs 8.1%; P = .034), more frequently underwent Crawford extent III repair (56.5% vs 34.8%; P = .002) and applied normothermic iliac perfusion (91.3% vs 81.4%; P = .057). There was no significant difference in the rate of early composite adverse events between the MFS and non-MFS groups (23.2% vs 14.3%; P = .099), which was verified by multivariable logistic regression analyses with multiple models. Overall mortality was significantly lower in the MFS group compared to the non-MFS group (P = .026, log-rank test), with 1-, 5-, and 10-year cumulative mortality of 4.4% versus 8.7%, 8.1% versus 17.2%, and 20.9% versus 36.4%, respectively. Multivariable Cox regression analyses across different models further confirmed MFS as a significant protective factor for overall mortality (model 1: hazard ratio [HR], 0.31; 95% confidence interval [CI] 0.13-0.73; P = .007; model 2: HR, 0.32, 95% CI, 0.13-0.75; P = .009; model 3: HR, 0.38; 95% CI, 0.15-0.95; P = .039).

CONCLUSIONS

Despite varying risk profiles, MFS patients undergoing open TAAA repair can achieve comparable or even superior outcomes to non-MFS patients with tailored surgical strategies, meticulous perioperative care, and close follow-up surveillance, especially in the long term.