Features of the Electrocardiogram in TMEM43 p.S358L Arrhythmogenic Cardiomyopathy
Abstract
BACKGROUND
The electrocardiogram (ECG) is important in the diagnostic evaluation of arrhythmogenic cardiomyopathy (ACM).
OBJECTIVE
The purpose of this study was to identify ECG features associated with TMEM43 p.S358L ACM over long-term assessment.
METHODS
A total of 634 ECGs were collected from a retrospective cohort of 68 patients with TMEM43 p.S358L ACM. Abnormalities in repolarization, depolarization, conduction intervals, QRS voltage, and rhythm were analyzed.
RESULTS
68 patients (32 males, 36 females) with ≥ 5 ECGs (median 9, range 5-17) over 20.5 ± 8.0 years were included. Over follow-up, 56/68 (82.4%) had an abnormality. Task Force Criteria (TFC) repolarization abnormalities were rare. Terminal activation duration ≥ 55 ms was common (20/32 males - 62.5%, 13/36 females - 36.1%). An epsilon wave was demonstrated in 2 female patients. Significant prolongation in the QRS duration occurred for males (97.3±11.6 ms to 137.6±24.8 ms, p <0.001) and females (90.4 ± 12.1 ms to 117.4 ±24.0ms, p <0.001). The most common finding was R wave < 3 mm in V3 (PRWP). Loss of the R wave in V3 < 3mm followed by development of an intraventricular conduction delay or complete left bundle branch block was seen in the majority of patients (42/68, 61.8%), occurring earlier in males than females (p <0.02).
CONCLUSION
Repolarization TFC 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 < 3mm and QRS prolongation…
How strong is the evidence?
Evidence Hub reads this as a cohort study, done in people. Its evidence level is 3 of 5 (Moderate · trial / cohort / secondary analysis). Trials without a large randomized design, cohorts and secondary analyses give moderate evidence. Reported size: 68 participants or records.
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