Heart failure & cardiomyopathies

Features of the electrocardiogram in TMEM43 p.S358L arrhythmogenic cardiomyopathy

Heart RhythmPublished retrospective cohort

Authors' conclusion

Repolarization Task Force criteria and epsilon waves are rare in TMEM43 p.S358L ACM. The ECG in TMEM43 p.S358L ACM is characterized by loss of the R wave in V3 to <3 mm and QRS prolongation with development of an intraventricular conduction delay or left bundle branch block.

Study context

Population
Patients with TMEM43 p.S358L arrhythmogenic cardiomyopathy (ACM) with ≥5 electrocardiogram (ECGs) during long-term assessmentpatients (32 male, 36 female), 634 ECGs
Participants
68 patients
Exposure
Male patients vs female patients
  • Male patientsn = 32
  • Female patientsn = 36
Outcome
Any ECG abnormality
Follow-up
During

Main finding

As reported in the abstract

Had an abnormality

56 of 68

82.4%

Observational data: associations, not effects.

Further findings

Terminal activation duration ≥55 ms

OutcomeMale patientsFemale patients
QRS duration prolongation97.3 ± 11.6 ms to 137.6 ± 24.8 ms90.4 ± 12.1 ms to 117.4 ± 24.0 ms
Loss of the R wave in V3 <3 mm followed by intraventricular conduction delay or complete left bundle branch block — seen in most patients · occurring earlier in male than in female patients
42/68 [61.8%] · P < 0.02
  • QRS duration prolongation: male patients P < .001
  • QRS duration prolongation: female patients P < .001

Task Force criteria repolarization abnormalities

Task Force criteria repolarization abnormalities were rare

Epsilon wave

An epsilon wave was demonstrated in female patients
2

R wave <3 mm in V3 (poor R-wave progression)

The most common finding was R wave <3 mm in V3 (poor R-wave progression)

Original publication

Features of the electrocardiogram in TMEM43 p.S358L arrhythmogenic cardiomyopathy. Heart Rhythm. Published 20 March 2025. doi:10.1016/j.hrthm.2025.03.1965

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