An evaluation of 24 h Holter monitoring in patients with myotonic dystrophy type 1

I.B.T. Joosten*, C.E.W. Janssen, C.G.C. Horlings, D. den Uijl, R. Evertz, B.G.M. van Engelen, C.G. Faber, K. Vernooy

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Aims To evaluate the clinical effectiveness of routine 24 h Holter monitoring to screen for conduction disturbances and arrhythmias in patients with myotonic dystrophy type 1 (DM1). Methods and results A retrospective two-centre study was conducted including DM1-affected individuals undergoing routine cardiac screening with at least one 24 h Holter monitoring between January 2010 and December 2020. For each individual, the following data were collected: Holter results, results of electrocardiograms (ECGs) performed at the same year as Holter monitoring, presence of cardiac complaints, and neuromuscular status. Holter findings were compared with the results of cardiac screening (ECG + history taking) performed at the same year. Cardiac conduction abnormalities and/or arrhythmias that would have remained undiagnosed based on history taking and ECG alone were considered de novo findings. A total 235 genetically confirmed DM1 patients were included. Abnormal Holter results were discovered in 126 (54%) patients after a mean follow-up of 64 +/- 28 months in which an average of 3 +/- 1 Holter recordings per patient was performed. Abnormalities upon Holter mainly consisted of conduction disorders (70%) such as atrioventricular (AV) block. Out of 126 patients with abnormal Holter findings, 74 (59%) patients had de novo Holter findings including second-degree AV block, atrial fibrillation/flutter and non-sustained ventricular tachycardia. Patient characteristics were unable to predict the occurrence of de novo Holter findings. In 39 out of 133 (29%) patients with normal ECGs upon yearly cardiac screening, abnormalities were found on Holter monitoring during follow-up. Conclusion Twenty-four hour Holter monitoring is of added value to routine cardiac screening for all DM1 patients.
Original languageEnglish
Pages (from-to)156-163
Number of pages8
JournalEP Europace
Issue number1
Early online date19 Jul 2022
Publication statusPublished - 8 Feb 2023


  • Myotonic dystrophy
  • Neuromuscular disease
  • Electrocardiogram
  • Holter monitoring
  • Ambulatory monitoring


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