Heart rhythm · 2025
Features of the electrocardiogram in TMEM43 p.S358L arrhythmogenic cardiomyopathy.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Duffett Stephen A, Compton Chris, Vasanthan Kieran, Short Ian, Gill Harinder Pal, Young Terry-Lynn, Connors Sean P, Hodgkinson Kathleen A
Аннотация
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 V3 (poor R-wave progression). Loss of the R wave in V3 <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 V3 to <3 mm and QRS prolongation with development of an intraventricular conduction delay or left bundle branch block.
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 V3 (poor R-wave progression). Loss of the R wave in V3 <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 V3 to <3 mm and QRS prolongation with development of an intraventricular conduction delay or left bundle branch block.