In patients with Marfan syndrome, a preoperative left ventricular end-diastolic dimension ≥65 mm was independently associated with an increased risk of all-cause mortality (HR 2.39).
Cohort (n=67)
No
Does preoperative LVEDD ≥65 mm or surgical intervention affect all-cause mortality in patients with Marfan syndrome and aortic aneurysm or dissection?
In patients with Marfan syndrome, a preoperative LVEDD ≥65 mm is an independent predictor of long-term mortality, suggesting it may serve as a complementary risk stratification marker beyond aortic diameter.
Hazard Ratio: 2.39 (95% CI 1.06–5.38)
p-value: p=0.035
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.
Cheng et al. (Sat,) conducted a cohort in Marfan syndrome with aortic aneurysm or dissection (n=67). Preoperative left ventricular end-diastolic dimension (LVEDD) ≥65 mm vs. LVEDD <65 mm was evaluated on All-cause mortality (HR 2.39, 95% CI 1.06-5.38, p=0.035). In patients with Marfan syndrome, a preoperative left ventricular end-diastolic dimension ≥65 mm was independently associated with an increased risk of all-cause mortality (HR 2.39).
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