ArticleClinical research in cardiology : official journal of the German Cardiac Society2019
Non-invasive evaluation of the relationship between electrical and structural cardiac abnormalities in patients with myotonic dystrophy type 1.
Article in Clinical research in cardiology : official journal of the German Cardiac Society, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed.
- Role of cardiovascular magnetic resonance in diagnosis and management of muscular dystrophies.Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance · 2026Review
- Atlas of Regional Left Ventricular Scar in Nonischemic Cardiomyopathies: Substrates and Etiologies.JACC. Advances · 2024Review
- Comprehensive Cardiovascular Management of Myotonic Dystrophy Type 1 Patients: A Report from the Italian Neuro-Cardiology Network.Journal of cardiovascular development and disease · 2024Review
- Cardiac risk and myocardial fibrosis assessment with cardiac magnetic resonance in patients with myotonic dystrophy.Frontiers in neurology · 2024Article
- Prognostic Utility of Cardiovascular Magnetic Resonance-Based Phenotyping in Patients With Muscular Dystrophy.Journal of the American Heart Association · 2023Article
- Neuromuscular diseases and their cardiac manifestations under the spectrum of cardiovascular imaging.Heart failure reviews · 2022Review
- Asymptomatic myocardial infarction in a patient with myotonic dystrophy type 1.Journal of cardiology cases · 2022Article
- Cardiac Involvement and Arrhythmias Associated with Myotonic Dystrophy.Reviews in cardiovascular medicine · 2022Article
- Cardiac Pathology in Myotonic Dystrophy Type 1.International journal of molecular sciences · 2021Review
- Metabolic Alterations in Myotonic Dystrophy Type 1 and Their Correlation with Lipin.International journal of environmental research and public health · 2021Review
- Systemic therapy in an RNA toxicity mouse model with an antisense oligonucleotide therapy targeting a non-CUG sequence within the DMPK 3'UTR RNA.Human molecular genetics · 2020Article
- Rare Disease: Cardiac Risk Assessment With MRI in Patients With Myotonic Dystrophy Type 1.Frontiers in neurology · 2020Article
- The Added Value of Cardiac Magnetic Resonance in Muscular Dystrophies.Journal of neuromuscular diseases · 2019Review
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Authors and funding
7 authors.
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Abstract
backgroundCardiac 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.
methodsFifty-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.
resultsAbnormal 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.
conclusionMyocardial 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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