Impact of the T-wave characteristics on distinguishing arrhythmogenic right ventricular cardiomyopathy from healthy children
Introduction
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited progressive myocardial disease characterized by fibro-fatty replacement in the right ventricle (RV). ARVC typically becomes manifest with ventricular arrhythmias during the patients' twenties to forties, sometimes causing sudden death [1,2]. Pediatric cases are rare due to age-related penetrance, but those patients are reported to have a higher risk of life-threating arrhythmias than adult patients [3].
The diagnosis of ARVC is generally made by the revised Task Force Criteria (rTFC), including 12‑lead electrocardiograms (ECGs), Holter ECGs, signal-averaged ECGs (SAECGs), echocardiography, cardiac magnetic resonance imaging (CMR), angiography, and a detailed family history [4]. However, most of those findings may become manifest after an advanced state of the disease [5,6], therefore pediatric asymptomatic patients have the risk of being overlooked.
Because the ECG is one of the easiest, non-invasive, and low-cost examinations, it is important for screening for ARVC [7]. Although T-wave inversion (TWI) in right precordial leads is adopted in the rTFC, it is generally recognized as a normal juvenile pattern in children, therefore, this finding sometimes confuses the diagnosis of pediatric ARVC patients. To prevent this confusion, the rTFC does not include TWI in children less than 14 years old, but ARVC children with fewer findings other than TWI may be overlooked. Prior studies have focused on the prevalence of TWI between ARVC patients and healthy adults [[8], [9], [10]] or young athletes [11,12], but there have been no reports comparing the TWI characteristics between ARVC children and healthy children.
In Japan, for the purpose of an early detection of heart diseases, school heart screening is established as a national system, and is performed annually for students in the 1st grade in elementary school (6 years old), 7th grade in junior high school (12 years old), and 10th grade in high school (15 years old). It consists of simple, convenient, and cost-effective examinations such as questionnaires, physician's assessments, and ECGs. The current ECG criteria for the Japanese primary screening have been described in a previous paper [13], and 1) TWI ≥ 0.1 mV in leads I, II, aVL, aVF, or V3 to V6, 2) a notch (ε-wave) after the QRS waves in leads V1–V3, and 3) the presence of left bundle branch block (LBBB) type ventricular tachycardias (VTs) or premature ventricular contractions (PVCs), have been applied for ARVC [13]. However pediatric ARVC patients rarely exhibit characteristic findings such as ε-waves or VTs [14], hence, there would be a great advantage if we could suspect the presence of ARVC with a TWI pattern during the primary screening.
The present study aimed to clarify the difference of TWI characteristics between ARVC and healthy children, and to predict the severity on the basis of the ECG.
Section snippets
Study population
This study was a retrospective observation study of ARVC patients before the age of 18. We reviewed the Japanese Pediatric Cardiomyopathy Registry, which enrolled 11 consecutive Asian subjects (eight males) who were diagnosed with ARVC at multi-centers in Japan from 1979 to 2017. The mean age at the time of the initial ECG was 13.3 ± 4.7 years, and the mean follow-up period was 3.3 ± 2.0 years. We included patients into the registry if they were classified as “Definite” on the basis of the 2010
Clinical features
All the patients were diagnosed as “Definite” based on the rTFC (Table 1). Asymptomatic patients (9 patients, 81.8%) were detected by school screening. Abnormalities in the ECGs included LBBB type PVCs (n = 3), TWI in the precordial and inferior leads (n = 4), and atrioventricular block (n = 2). Two remaining patients had chest pain and a fetal echo abnormality. Three patients experienced an aSCD during exercise at school at ages of 17, 14, and 18 respectively (Pt. 2, 3, 8). Pt. 11 had syncope.
Discussion
This study clarified distinct ECG differences in ARVC children from healthy children. Our main findings were that TWD was rare in healthy children, and it was a specific and sensitive sign for ARVC children, especially for symptomatic patients. To the best of our knowledge, this is the first report to clarify that TWD is a valid sign for distinguishing ARVC from healthy children, and predicting the severity of ARVC.
Limitations
Several limitations of this study should be acknowledged. First, because of the small sample size, there was a possibility that some findings were determined to have no significant difference. Moreover, it was not definitive whether or not TWD and anterior TWI were useful in predicting the severity because of the small number of patients. Second, because the duration of the follow-up was limited to 3.3 years, we could not comment on the long-term outcomes of pediatric ARVC. Third, because all
Conclusions
To the best of our knowledge, the present study was the first study to clarify that TWD is associated with ARVC. TWD and anterior TWI had the potential to distinguish between healthy children and ARVC children, especially those who may develop severe symptoms in the future. Simple, non-invasive, and cost-effective ECG screening may enable early interventions to prevent SCD in pediatric ARVC patients.
Author statement
This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.
Acknowledgement of grant support
This work was supported by the Japanese Health and Labour Science Research for Dr. Yoshinaga [grant number H27-019]. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
Declaration of Competing Interest
None.
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