Ventricular dispersion and repolarization in Marfan syndrome: A clinical analysis

Patients with Marfan syndrome (MFS) are at an increased risk of ventricular arrythmia (VA) and death.

This retrospective observational study aimed to assess the role of electrocardiographic parameters of repolarization and dispersion in risk stratification.

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.

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.

Electrocardiographic parameters of repolarization and dispersion are altered in patients with MFS, but their role in risk stratification may be limited.