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

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.

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.

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).

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.