Key result
Repolarization abnormalities in Marfan syndrome show no association with ventricular arrhythmias or mortality.
Why the study?
Patients with Marfan syndrome are at increased risk of ventricular arrhythmia and death, prompting an evaluation of electrocardiographic repolarization and dispersion parameters for risk stratification.
Do electrocardiographic parameters of repolarization and dispersion predict ventricular arrhythmias or mortality in patients with Marfan syndrome?
Observational (n=181)
No
Do electrocardiographic parameters of repolarization and dispersion predict ventricular arrhythmias or mortality in patients with Marfan syndrome?
Hazard Ratio: 1.02 (95% CI 0.98–1.05)
p-value: p=0.320
Despite finding altered repolarization and dispersion in Marfan syndrome, routine ECG measures do not reliably predict ventricular arrhythmias or mortality, highlighting the need for alternative risk stratification approaches.
ECG repolarization measures lack prognostic value in Marfan syndrome; leaves open alternative risk stratification approaches.
Background: Patients with Marfan syndrome (MFS) are at an increased risk of ventricular arrythmia (VA) and death. Objective: This retrospective observational study aimed to assess the role of electrocardiographic parameters of repolarization and dispersion in risk stratification. Methods: Baseline 12-lead electrocardiograms were obtained from consecutive patients with MFS treated at a tertiary care specialized outpatient clinic and from age- and sex-matched controls. In patients with MFS, we studied the association of established parameters of repolarization and dispersion with a combined primary end point of VA or all-cause mortality using Cox regression analysis. Results: A total of 89 patients with MFS (mean age 42 ± 14 years; 54 (61%) women) and 92 controls were included in the analysis. The mean QRS duration (97 ms vs 87 ms) was longer, and the mean corrected QT interval (411 ms vs 379 ms) and T-peak-T-end interval (67 ms vs 62 ms) were also longer in patients with MFS. During a median follow-up of 5.05 years (interquartile range 3.43-10.0 years), 11 patients with MFS (12%) had VA or died. In univariable analyses, male sex (hazard ratio [HR] 5.01; 95% confidence interval [CI] 1.051-23.87), history of atrial fibrillation (HR 9.51; 95% CI 2.51-36.01), larger left atrial volume (HR 1.35 per 10-mL increase; 95% CI 1.12-1.64 per 10-mL increase), and higher N-terminal pro-B-type brain natriuretic peptide levels (HR 1.05 per 100-pg/mL increase; 95% CI 1.03-1.07 per 100-pg/mL increase) were associated with the primary end point, but QRS duration, corrected QT interval, and T-peak-T-end interval or their derivatives were not associated with the primary end point. Conclusion: Electrocardiographic parameters of repolarization and dispersion are altered in patients with MFS, but their role in risk stratification may be limited.
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Spittler et al. (2025) conducted an observational in Marfan syndrome (n=181). Electrocardiographic parameters of repolarization and dispersion (e.g., QRS duration, QTc, TpTe) vs. Age- and sex-matched controls (for baseline ECG comparison); intra-cohort comparison for outcome prediction was evaluated on Combined end point of ventricular arrhythmia or all-cause mortality (HR 1.02, 95% CI 0.98-1.05, p=0.320). Although electrocardiographic parameters of repolarization and dispersion are altered in patients with Marfan syndrome compared to controls, they were not significantly associated with ventricular arrhythmias or all-cause mortality.
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