ArticleJournal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance2014
Myocardial fibrosis in patients with myotonic dystrophy type 1: a cardiovascular magnetic resonance study.
Article in Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it, 54 citations in OpenAlex.
- Clinical Care Recommendations for Cardiologists Treating Adults With Myotonic Dystrophy.Journal of the American Heart Association · 2020Guideline
- 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
- Late gadolinium enhancement imaging and sudden cardiac death.European heart journal · 2025Review
- Evaluation of echocardiography monitoring in myotonic dystrophy type 1 patients.Frontiers in cardiovascular medicine · 2025Article
- 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 Involvement in LAMA2-Related Muscular Dystrophy and SELENON-Related Congenital Myopathy: A Case Series.Journal of neuromuscular diseases · 2024Article
- 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
- Periostin as a blood biomarker of muscle cell fibrosis, cardiomyopathy and disease severity in myotonic dystrophy type 1.Journal of neurology · 2023Article
- Neuromuscular diseases and their cardiac manifestations under the spectrum of cardiovascular imaging.Heart failure reviews · 2022Review
- A rare case report of severe cardiomyopathy associated with myotonic dystrophy type 2.European heart journal. Case reports · 2022Article
- Diagnostic and Prognostic Value of Cardiovascular Magnetic Resonance in Neuromuscular Cardiomyopathies.Pediatric cardiology · 2022Article
- Cardiac Pathology in Myotonic Dystrophy Type 1.International journal of molecular sciences · 2021Review
- Cardiac Involvement in Women With Pathogenic Dystrophin Gene Variants.Frontiers in neurology · 2021Article
- Cardiac involvement in a cross-sectional cohort of myotonic dystrophies and other skeletal myopathies.ESC heart failure · 2020Article
- 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
- Myotonic dystrophy type 1 and high ventricular vulnerability at the electrophysiological evaluation: ICD yes or not?Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology · 2020Article
- Rare Disease: Cardiac Risk Assessment With MRI in Patients With Myotonic Dystrophy Type 1.Frontiers in neurology · 2020Article
- Advancements in magnetic resonance imaging-based biomarkers for muscular dystrophy.Muscle & nerve · 2019Review
- Non-invasive evaluation of the relationship between electrical and structural cardiac abnormalities in patients with myotonic dystrophy type 1.Clinical research in cardiology : official journal of the German Cardiac Society · 2019Article
Corrections and comments
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMyotonic dystrophy type 1 (DM1) is associated with increased cardiac morbidity and mortality. Therefore, assessment of cardiac involvement and risk stratification for sudden cardiac death is crucial. Nevertheless, optimal screening-procedures are not clearly defined. ECG, echocardiography and Holter-monitoring are useful but insufficient. Cardiovascular magnetic resonance (CMR) can provide additional information of which myocardial fibrosis may be relevant. The purpose of this study was to describe the prevalence of myocardial fibrosis in patients with DM1 assessed by CMR, and the association between myocardial fibrosis and abnormal findings on ECG, Holter-monitoring and echocardiography.
methodsWe selected 30 unrelated patients with DM1: 18 patients (10 men, mean age 51 years) with, and 12 patients (7 men, mean age 41 years) without abnormal findings on ECG and Holter-monitoring. Patients were evaluated with medical history, physical examination, ECG, Holter-monitoring, echocardiography and CMR.
resultsMyocardial fibrosis was found in 12/30 (40%, 9 men). The presence of myocardial fibrosis was associated with the following CMR-parameters: increased left ventricular mass (median (range) 55 g/m² (43-83) vs. 46 g/m² (36-64), p = 0.02), increased left atrial volume (median (range) 52 ml/m² (36-87) vs. 46 ml/m² (35-69), p = 0.04) and a trend toward lower LVEF (median (range) 63% (38-71) vs. 66% (60-80), p = 0.06). Overall, we found no association between the presence of myocardial fibrosis and abnormal findings on: ECG (p = 0.71), Holter-monitoring (p = 0.27) or echocardiographic measurements of left ventricular volumes, ejection fraction or global longitudinal strain (p = 0.18).
conclusionPatients with DM1 had a high prevalence of myocardial fibrosis which was not predicted by ECG, Holter-monitoring or echocardiography. CMR add additional information to current standard cardiac assessment and may prove to be a clinically valuable tool for risk stratification in DM1.
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