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Features of the electrocardiogram in TMEM43 p.S358L arrhythmogenic cardiomyopathy

Stephen A Duffett1, Chris Compton1, Kieran Vasanthan1

  • 1Division of Cardiology, Faculty of Medicine, Memorial University of Newfoundland, St John's, Newfoundland and Labrador, Canada.

Heart Rhythm
|March 22, 2025
PubMed

Insights

Electrocardiograms (ECGs) in TMEM43 p.S358L arrhythmogenic cardiomyopathy (ACM) show QRS prolongation and loss of R wave in V3. These ECG changes, including intraventricular conduction delay, are key diagnostic indicators.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Diagnostics

Background:

  • The electrocardiogram (ECG) is crucial for diagnosing arrhythmogenic cardiomyopathy (ACM).
  • Specific ECG features associated with genetic variants like TMEM43 p.S358L ACM require detailed long-term assessment.

Purpose of the Study:

  • To identify and characterize electrocardiogram (ECG) features in patients with TMEM43 p.S358L arrhythmogenic cardiomyopathy (ACM).
  • To analyze the long-term evolution of ECG abnormalities in this specific ACM population.

Main Methods:

  • Retrospective analysis of 634 ECGs from 68 patients diagnosed with TMEM43 p.S358L ACM.
  • Evaluation of repolarization, depolarization, conduction intervals, QRS voltage, and rhythm abnormalities over a median follow-up of 9 ECGs per patient.
  • Long-term assessment spanning 20.5 ± 8.0 years.

Main Results:

  • Significant QRS duration prolongation was observed in both male and female patients (P < .001).
  • Loss of R wave in V3 (poor R-wave progression) followed by intraventricular conduction delay or left bundle branch block occurred in 61.8% of patients, earlier in males.
  • Repolarization abnormalities and epsilon waves were rare; terminal activation duration ≥55 ms was common in males (62.5%).

Conclusions:

  • ECG findings in TMEM43 p.S358L ACM are primarily characterized by QRS prolongation and loss of R wave in V3.
  • Development of intraventricular conduction delay or left bundle branch block is a significant ECG progression in this condition.
  • Standard repolarization criteria and epsilon waves are infrequent, highlighting the need for specific ECG pattern recognition in TMEM43 p.S358L ACM.
Abstract

Keywords:
Arrhythmogenic cardiomyopathyElectrocardiographyEvolutionGeneticsTMEM43

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