Features of the electrocardiogram in TMEM43 p.S358L arrhythmogenic cardiomyopathy
Bibliographic record
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 during 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 Sixty-eight patients (32 male, 36 female) with ≥5 ECGs (median, 9; range, 5–17) during 20.5 ± 8.0 years were included. During follow-up, 56 of 68 (82.4%) had an abnormality. Task Force criteria repolarization abnormalities were rare. Terminal activation duration ≥55 ms was common (20/32 male patients [62.5%]; 13/36 female patients [36.1%]). An epsilon wave was demonstrated in 2 female patients. Significant prolongation in the QRS duration occurred for male patients (97.3 ± 11.6 ms to 137.6 ± 24.8 ms; P < .001) and female patients (90.4 ± 12.1 ms to 117.4 ± 24.0 ms; P < .001). The most common finding was R wave <3 mm in V 3 (poor R-wave progression). Loss of the R wave in V 3 <3 mm followed by development of an intraventricular conduction delay or complete left bundle branch block was seen in most patients (42/68 [61.8%]), occurring earlier in male than in female patients ( P < .02). 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 V 3 to <3 mm and QRS prolongation with development of an intraventricular conduction delay or left bundle branch block.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".