ORIGINAL RESEARCH article

Front. Cardiovasc. Med.

Sec. Cardiovascular Imaging

Volume 12 - 2025 | doi: 10.3389/fcvm.2025.1574885

Evaluation of echocardiography monitoring in myotonic dystrophy type 1 patients

Provisionally accepted
David  S.H. BovenkerkDavid S.H. Bovenkerk1,2*Cheyenne  E.W. JanssenCheyenne E.W. Janssen1Frederik  M.A. Van den HeuvelFrederik M.A. Van den Heuvel3Isis  B.T. JoostenIsis B.T. Joosten1Dennis  Den UijlDennis Den Uijl2Baziel  G.M. Van EngelenBaziel G.M. Van Engelen4Sander  M.J. Van KuijkSander M.J. Van Kuijk5Robin  NijveldtRobin Nijveldt3Reinder  EvertzReinder Evertz3Catharina  G. FaberCatharina G. Faber1Kevin  VernooyKevin Vernooy2Geertruida  P. BijvoetGeertruida P. Bijvoet2
  • 1Department of Neurology, Maastricht University Medical Centre, Maastricht, Netherlands, Netherlands
  • 2Department of Cardiology, Cardiovascular Research Institute Maastricht (CARIM), Maastricht University Medical Center+, Maastricht, Netherlands
  • 3Department of Cardiology, Radboud University Medical Centre, Nijmegen, Netherlands
  • 4Department of Neurology, Radboud University Medical Centre, Nijmegen, Gelderland, Netherlands
  • 5Department of Clinical Epidemiology and Medical Technology Assessment, Maastricht University Medical Centre, Maastricht, Netherlands, Netherlands

The final, formatted version of the article will be published soon.

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.This retrospective cohort study with DM1 patients was conducted in Maastricht University Medical Center (MUMC+) and Radboud University Medical center. All patients above 18

Keywords: Myotonic dystrophy type 1, Echocardiography, Left ventricular dysfunction, Routine echocardiographic follow-up, Conduction abnormalities

Received: 11 Feb 2025; Accepted: 16 Jun 2025.

Copyright: © 2025 Bovenkerk, Janssen, Van den Heuvel, Joosten, Den Uijl, Van Engelen, Van Kuijk, Nijveldt, Evertz, Faber, Vernooy and Bijvoet. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: David S.H. Bovenkerk, Department of Neurology, Maastricht University Medical Centre, Maastricht, 6229 HX, Netherlands, Netherlands

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