Features of the Electrocardiogram in TMEM43 p.S358L Arrhythmogenic Cardiomyopathy.

IF 5.6 2区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

Heart rhythm Pub Date : 2025-03-20 DOI:10.1016/j.hrthm.2025.03.1965

Stephen A Duffett, Chris Compton, Kieran Vasanthan, Ian Short, Harinder Pal Gill, Terry-Lynn Young, Sean P Connors, Kathleen A Hodgkinson

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引用次数: 0

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 with development of an IVCD or LBBB.

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来源期刊

Heart rhythm 医学-心血管系统

CiteScore

10.50

自引率

5.50%

发文量

1465

审稿时长

24 days

期刊介绍： HeartRhythm, the official Journal of the Heart Rhythm Society and the Cardiac Electrophysiology Society, is a unique journal for fundamental discovery and clinical applicability. HeartRhythm integrates the entire cardiac electrophysiology (EP) community from basic and clinical academic researchers, private practitioners, engineers, allied professionals, industry, and trainees, all of whom are vital and interdependent members of our EP community. The Heart Rhythm Society is the international leader in science, education, and advocacy for cardiac arrhythmia professionals and patients, and the primary information resource on heart rhythm disorders. Its mission is to improve the care of patients by promoting research, education, and optimal health care policies and standards.