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Updated: Jan 29, 2026
Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
Published on: July 29, 2016
Non-invasive evaluation of the relationship between electrical and structural cardiac abnormalities in patients with
Lukas Chmielewski1, Michael Bietenbeck1, Alexandru Patrascu2
1Department of Cardiology I, University Hospital Muenster, Münster, Germany.
Insights
In myotonic dystrophy type 1 (MD1), non-ischemic late gadolinium enhancement (LGE) on cardiac MRI independently predicts atrial fibrillation/flutter. This finding aids in predicting adverse cardiac events in MD1 patients.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Myotonic dystrophy type 1 (MD1) cardiac involvement includes conduction disease, arrhythmias, and left-ventricular (LV) dysfunction, increasing sudden cardiac death risk.
- Understanding the interplay between electrical and structural myocardial changes is crucial for predicting adverse cardiac events in MD1.
Purpose of the Study:
- To explore the relationship between cardiac magnetic resonance (CMR) findings and electrical abnormalities in MD1 patients.
- To determine if advanced CMR techniques can predict arrhythmias in MD1.
Main Methods:
- Fifty-seven MD1 patients and 15 controls underwent CMR, including cine-imaging, feature-tracking strain analysis, late gadolinium enhancement (LGE), and T1-mapping.
- Standard and long-term ECG monitoring were performed to screen for rhythm and conduction abnormalities.
Main Results:
- Abnormal ECGs were found in 40% of MD1 patients; 44% had abnormal CMR findings, including impaired LV ejection fraction (EF) and non-ischemic LGE.
- MD1 patients with atrial fibrillation/flutter episodes showed significantly lower LV-EF, lower global longitudinal strain, and a higher prevalence of LGE, particularly with intramural and septal patterns.
- Late gadolinium enhancement (LGE) presence was the only independent predictor of atrial fibrillation/flutter episodes (OR 14.8, p=0.026).
Conclusions:
- Non-ischemic myocardial abnormalities detected by LGE-CMR are the sole independent predictor of atrial fibrillation/flutter on ECG monitoring in MD1.
- These findings highlight the importance of LGE-CMR in assessing cardiac risk and predicting adverse events in MD1.
Background:
Cardiac involvement in myotonic dystrophy type 1 (MD1) includes conduction disease, arrhythmias, and left-ventricular (LV) systolic dysfunction leading to an increased sudden cardiac death risk. An understanding of the interplay between electrical and structural myocardial changes could improve the prediction of adverse cardiac events. We aimed to explore the relationship between signs of cardiomyopathy by conventional and advanced cardiovascular magnetic resonance (CMR), and electrical abnormalities in MD1.
Methods:
Fifty-seven MD1 patients (43 ± 13 years, 46% male) and 15 matched controls (41 ± 7 years, 53% male) underwent CMR including cine-imaging with feature-tracking strain analysis, late gadolinium enhancement (LGE), and native/post-contrast T1-mapping with extracellular volume calculation. Standard 12-lead and long-term ECG monitoring were performed as screening for rhythm and/or conduction abnormalities.
Results:
Abnormal ECGs were recorded in 40% of MD1; a pathologic CMR was found in 44%: 21% had an impaired LV-EF and 32% showed non-ischemic LGE. When looking at MD1 patients with available long-term ECG monitoring (n = 39), those with atrial fibrillation (Afib)/flutter(Afl) episodes had lower LV-EF (52 ± 7 vs. 60 ± 5%, p = 0.002), lower global longitudinal strain (- 17 ± 3 vs. - 20 ± 3%, p = 0.034), a trend to lower left atrial emptying fraction (LA-EF) (44 ± 14 vs. 55 ± 8%, p = 0.08), and higher prevalence of LGE (88% vs. 23%, p = 0.001) with an intramural (75% vs. 23%, p = 0.01) and septal (63% vs. 13%, p = 0.009) pattern. In a model including LV-EF (OR 0.8, 95% CI 0.7-1.0, p = NS) and LGE presence (OR 14.8, 95% CI 1.4-159.0, p = 0.026), only LGE was independently associated with the occurrence of Afib/Afl episodes.
Conclusion:
Myocardial abnormalities depicted by non-ischemic LGE-CMR were the only independent predictor for the occurrence of Afib/Afl on ECG monitoring, previously shown to predict adverse cardiac events in MD1.
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