Heart failure & cardiomyopathies
Features of the electrocardiogram in TMEM43 p.S358L arrhythmogenic cardiomyopathy
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
| Outcome | Male patients | Female patients |
|---|---|---|
| QRS duration prolongation | 97.3 ± 11.6 ms to 137.6 ± 24.8 ms | 90.4 ± 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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