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Lookup NU author(s): Dr Zdenka Reinhardt
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology. AIMS: The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. METHODS AND RESULTS: Data from 356 childhood HCM patients with a mean age of 10.1 years (±4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0-7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93-2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of >5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484-0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7. CONCLUSION: In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited.
Author(s): Norrish G, Topriceanu C, Qu C, Field E, Walsh H, Ziolkowska L, Olivotto I, Passantino S, Favilli S, Anastasakis A, Vlagkouli V, Weintraub R, King I, Biagini E, Ragni L, Prendiville T, Duignan S, McLeod K, Ilina M, Fernandez A, Bokenkamp R, Baban A, Drago F, Kubus P, Daubeney PEF, Chivers S, Sarquella-Brugada G, Cesar S, Marrone C, Medrano C, Alvarez Garcia-Roves R, Uzun O, Gran F, Castro FJ, Gimeno JR, Barriales-Villa R, Rueda F, Adwani S, Searle J, Bharucha T, Siles A, Usano A, Rasmussen TB, Jones CB, Kubo T, Mogensen J, Reinhardt Z, Cervi E, Elliott PM, Omar RZ, Kaski JP
Publication type: Article
Publication status: Published
Journal: European Journal of Preventive Cardiology
Year: 2022
Volume: 29
Issue: 4
Pages: 645-653
Print publication date: 01/03/2022
Online publication date: 27/03/2021
Acceptance date: 09/03/2021
Date deposited: 28/04/2022
ISSN (print): 2047-4873
ISSN (electronic): 2047-4881
Publisher: Oxford University Press
URL: https://doi.org/10.1093/eurjpc/zwab046
DOI: 10.1093/eurjpc/zwab046
PubMed id: 33772274
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