In order to prevent sudden cardiac death in patients with DM1, cardiologists recommend the implantation of a pacemaker in asymptomatic patients with specific abnormalities detectable by electrocardiogram (ECG) or electrophysiological study (EPS).
In order to determine which strategy, based on ECG or EPS, is most effective in predicting major bradyarrhythmic events (MBAE) in patients, French researchers, including clinicians from the Institute’s service of Neuro-Myology, conducted a retrospective longitudinal study. The data came from 706 genetically confirmed DM1 patients (359 men and 347 women, aged 29 to 55) from the DM1 Heart Registry, over the period January-July 2025.
In this cohort of DM1 patients, the EPS criterion proved to be more reliable and effective than ECG criteria in predicting MBAE. An HV threshold greater than or equal to 65 milliseconds could improve risk stratification for prophylactic pacing.
