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Electrocardiogram vs Electrophysiological Study and Major Conduction Delays in Myotonic Dystrophy Type 1
Nicolas Clementy1,2, Fabien Labombarda3, François Grolleau4,5,6
1Department of Cardiology, Université de Tours, CHU de Tours, Paris, France.
JAMA Cardiology
|September 24, 2025
Summary
In myotonic dystrophy type 1 (DM1), His-ventricular (HV) interval measurement during electrophysiological study (EPS) is more effective than electrocardiogram (ECG) criteria for predicting major bradyarrhythmic events (MBAEs) and guiding pacemaker implantation.
Area of Science:
- Cardiology
- Electrophysiology
- Genetics
Background:
- Myotonic dystrophy type 1 (DM1) poses a risk of sudden cardiac death due to bradyarrhythmias.
- Current guidelines recommend pacemaker implantation based on ECG or EPS criteria in asymptomatic DM1 patients.
- The comparative effectiveness of ECG versus EPS in predicting major bradyarrhythmic events (MBAEs) requires further investigation.
Purpose of the Study:
- To compare the efficacy of ECG-based and EPS-based strategies in predicting MBAEs in DM1 patients.
- To evaluate the His-ventricular (HV) interval as a predictor of MBAEs.
- To determine an optimal HV interval threshold for risk stratification.
Main Methods:
- Retrospective cohort analysis of longitudinal data from the DM1 Heart Registry (2000-2020).
- Inclusion of adults with genetically confirmed DM1 who underwent their first EPS after 1999.
- Primary outcome: MBAEs (sudden cardiac death, cardiac arrest, advanced AV block).
Main Results:
- The His-ventricular (HV) interval was significantly associated with MBAE incidence (HR, 1.77; P<.001).
- EPS criteria were more reliable and sensitive predictors of MBAEs compared to ECG criteria.
- Lowering the HV interval threshold to ≥65 milliseconds improved sensitivity and reclassification of MBAE risk.
Conclusions:
- The HV interval measured during EPS is a superior predictor of MBAEs in DM1 patients compared to ECG criteria.
- An HV interval threshold of ≥65 milliseconds may enhance risk stratification for prophylactic pacemaker implantation.
- EPS-based risk assessment offers improved prediction and patient management for sudden cardiac death prevention in DM1.
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