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Prevalence of cardiovascular implantable electronic devices in children with type 1 myotonic dystrophy

Omar Meziab1, Michael D Seckeler1, Katalin Scherer2

  • 1Department of Pediatrics (Cardiology), University of Arizona, Tucson, Arizona, USA.

Muscle & Nerve
|June 29, 2024
PubMed

Insights

Cardiovascular implantable electronic devices (CIEDs) are used in pediatric myotonic dystrophy type 1 (DM1) patients, despite limited data. This study found a 2.1% prevalence of CIEDs in pediatric DM1 patients, highlighting a need for specific guidelines.

Area of Science:

  • Neurology
  • Cardiology
  • Genetics

Background:

  • Type 1 myotonic dystrophy (DM1) is a multi-system disorder with significant electrophysiologic manifestations, including a 6.6% cumulative incidence of sudden death.
  • Genetic anticipation in DM1 creates a pediatric patient subset, yet cardiac care guidelines are primarily based on adult data.
  • This data gap complicates cardiac management for pediatric DM1 patients, often leading to the implantation of cardiovascular implantable electronic devices (CIEDs) based on adult protocols.

Purpose of the Study:

  • To investigate the prevalence of CIEDs in the pediatric DM1 population.
  • To compare CIED prevalence in pediatric DM1 patients versus adult DM1 patients.
  • To assess the odds of CIED implantation in pediatric and adult DM1 patients compared to non-DM1 populations.

Main Methods:

  • Utilized the Vizient® Clinical Data Base from October 2019 to October 2023.
  • Identified patients with myotonic dystrophy (ICD-10 code G71.11) and those with pacemakers or ICDs (Z95.0, Z95.810).
  • Stratified patients into Pediatric (0-21 years) and Adult (22-50 years) age groups for analysis.

Main Results:

  • The prevalence of CIEDs was 2.1% in pediatric DM1 patients and 15.8% in adult DM1 patients.
  • Pediatric DM1 patients had an odds ratio of 48.8 for CIED implantation compared to pediatric patients without DM1.
  • Adult DM1 patients had an odds ratio of 23.3 for CIED implantation compared to adult patients without DM1.

Conclusions:

  • Pediatric DM1 patients are receiving CIEDs without robust data to guide these decisions.
  • Further research is crucial to establish appropriate use of CIEDs in pediatric DM1.
  • Development of specific guidelines is needed to direct the management of cardiac care in this vulnerable population.
Abstract

Keywords:
ICDmyotonic dystrophypacemakerpediatrics

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