ArticleFrontiers in cardiovascular medicine2025
Evaluation of echocardiography monitoring in myotonic dystrophy type 1 patients.
Article in Frontiers in cardiovascular medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Introduction: Myotonic Dystrophy type 1 (DM1) is the most prevalent genetic neuromuscular disorder. The potential cardiac involvement, including arrhythmia and left ventricular dysfunction, demands routine follow-up with ECG and echocardiography, however recommendations on the interval of echocardiographic follow-up varies substantially. The aim of this study is to evaluate the long-term prevalence of LV dysfunction during echocardiographic follow-up of patients with DM1. Secondly, we aim to assess the association between structural abnormalities on echocardiography, ECG and clinical parameters. Methods: This retrospective cohort study with DM1 patients was conducted in Maastricht University Medical Center (MUMC+) and Radboud University Medical Center. All patients above 18 years old were included through the Dutch DM1 patient registry (MYODRAFT study). Patients were evaluated between January 2010 and December 2023. A total of 273 patients were included, in whom echocardiographic data was collected and correlated with clinical data, neuromuscular status and ECG parameters. Results: At baseline 20/273 (7.3%) patients had LV dysfunction (LVEF < 50% and/or LV dilatation). When newly detected LVEF <50% alone is considered then the yield of routine echocardiography follow-up was: 9/273 at baseline (3%), 2/84 at 36 months (2%), 9/184 at 72 months (5%) and 10/117 at the interval beyond 72 months (8%). The only clear correlation between ECG and echocardiography abnormalities was a widened QRS interval 125 ± 31 ms vs. 103 ± 19 ms ( Discussion: Based on the data of this large retrospective study, the occurrence of LV dysfunction in DM1 patients is rather low (7.4%) at baseline and increases with 6.5% at 72 months follow-up. There is a significant correlation between LV dysfunction and a widened QRS interval. Which could prompt earlier echocardiographic follow up in this patient group. Routine echocardiography is useful in DM1 however the interval of echocardiography could be shifted more towards 5 years in asymptomatic patients because of the slow progressive nature of the disease.
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