Prognostic factors in patients with Marfan syndrome referred to a cardiac surgery center: a retrospective cohort study.

BACKGROUND

Marfan syndrome (MFS) is associated with a high risk of aortic complications and premature mortality. This study aimed to evaluate long-term outcomes and identify clinical predictors of mortality in patients with MFS.

METHODS

We conducted a retrospective cohort study of consecutive patients diagnosed with MFS according to the revised Ghent criteria who presented between February 2000 and June 2022. Kaplan-Meier analysis was used to estimate overall survival, and survival was compared according to preoperative left ventricular end-diastolic dimension (LVEDD). Univariable and multivariable Cox regression analyses were performed to identify predictors of all-cause mortality. A time-dependent Cox regression model was additionally used to account for surgery as a time-varying covariate and reduce potential immortal time bias.

RESULTS

A total of 67 patients were included, with a mean age of 32 ± 10 years and a mean maximum ascending aortic diameter of 58.0 ± 11.7 mm. During a median follow-up of 53 months, the 1-, 5-, 10-, and 15-year overall survival rates were 74.6% (95% CI: 64.2-85.0), 62.8% (95% CI: 50.8-74.8), 48.7% (95% CI: 34.2-63.2), and 43.3% (95% CI: 27.0-59.6), respectively. Patients with preoperative LVEDD ≥ 65 mm had significantly worse survival (P = 0.024). In multivariable Cox regression analysis, preoperative LVEDD ≥65 mm (HR 2.39; 95% CI 1.06-5.38; P = 0.035) and emergent intubation (HR 6.43; 95% CI 1.54-26.88; P = 0.011) were independently associated with increased mortality, whereas surgical intervention was associated with lower mortality (HR 0.44; 95% CI 0.20-0.99; P = 0.046). In time-dependent Cox analysis, surgery remained significantly associated with lower mortality after adjustment (HR 0.31; 95% CI 0.13-0.70; P = 0.005).

CONCLUSION

In patients with MFS, larger LV dimensions, particularly LVEDD ≥65 mm, were associated with poorer long-term outcomes and may provide complementary information for exploratory risk stratification beyond aortic diameter. Surgical intervention was associated with lower mortality after accounting for time-dependent bias. These findings suggest that LVEDD may help identify high-risk patients and support its potential role as a complementary marker for risk stratification.