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Cardiac pacing in myotonic dystrophy type 1: a retrospective cohort study on indications, diagnostic modalities, and

Magnus Lans1, Linus Grundell1, Pär Parén1

  • 1Department of Medicine, Geriatrics and Emergency Care Mölndal, Sahlgrenska University Hospital, Gothenburg, Sweden; Department of Molecular and Clinical Medicine, Institution of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

International Journal of Cardiology
|July 17, 2026
PubMed

Insights

Patients with myotonic dystrophy type 1 (DM1) face sudden cardiac death risk. Cardiac screening with ECG and Holter monitoring helps identify needs for cardiac implantable electronic devices (CIEDs), with increasing pacing burden over time.

Area of Science:

  • Cardiology
  • Neuromuscular Disorders
  • Medical Devices

Background:

  • Myotonic dystrophy type 1 (DM1) patients have elevated risk of sudden cardiac death.
  • Cardiac screening is crucial for identifying DM1 patients who may benefit from cardiac pacing.
  • Cardiac implantable electronic device (CIED) implantation is a key management strategy.

Purpose of the Study:

  • To evaluate indications for CIED implantation in DM1 patients.
  • To assess diagnostic modalities used for screening DM1 patients.
  • To determine long-term ventricular pacing burden and mortality in DM1 patients.

Main Methods:

  • Retrospective observational cohort study of adults with DM1 in western Sweden.
  • Included patients referred for CIED implantation between 2000 and 2023.
  • Data collected on patient characteristics, ECG, Holter findings, pacing burden, and survival from electronic health records.

Main Results:

  • 102 patients underwent prophylactic CIED implantation.
  • ECG identified PR/QRS prolongation (Class IIb), Holter identified AV block (Class I).
  • Median follow-up was 5 years; ~33% had >40% ventricular pacing burden. All-cause mortality was 21%, primarily non-arrhythmic.

Conclusions:

  • Combined ECG and Holter screening offers complementary data for pacing indications in DM1.
  • Ventricular pacing burden shows a progressive increase over time.
  • Mortality in this cohort was mainly attributed to non-arrhythmic causes like respiratory insufficiency.
Abstract

Keywords:
Cardiac implantable electronic deviceCardiac screeningHolter monitoringMyotonic dystrophyPacemakerVentricular pacing

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