Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK.
Institute of Cardiovascular Sciences, University College London, London, UK.
Eur J Prev Cardiol. 2022 Mar 30;29(4):645-653. doi: 10.1093/eurjpc/zwab046.
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.
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.
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.
在患有肥厚型心肌病（HCM）的儿童中，通常会进行 12 导联心电图（ECG）检查。已经提出了心电图风险评分作为风险分层的有用工具，但尚未进行独立验证。本研究的目的是描述大型国际多中心队列中儿童 HCM 的心电图表型，并探讨其在心律失常事件风险预测中的作用。
从一个回顾性的、国际多中心的队列中收集了 356 名平均年龄为 10.1 岁（±4.5）的儿童 HCM 患者的数据。347 名（97.5%）患者在基线时有心电图异常，最常见的是复极异常（n=277，77.8%）；左心室肥厚（n=240，67.7%）；异常 QRS 轴（n=126，35.4%）或 QT 延长（n=131，36.8%）。在中位数为 3.9 年（四分位距 2.0-7.7）的随访中，25 名（7%）发生心律失常事件，总体年事件发生率为 1.38（95%置信区间 0.93-2.04）。在单变量或多变量分析中，没有心电图变量与 5 年心律失常事件相关。ECG 风险评分阈值>5 具有适度的区分能力[C 指数 0.60（95%置信区间 0.484-0.715）]，相应的阴性和阳性预测值分别为 96.7%和 6.7%。
在一个大型的国际多中心儿童 HCM 队列中，心电图异常很常见且多种多样。无论是孤立的还是以前描述的心电图风险评分中的组合，都没有与 5 年心源性猝死风险相关的心电图特征。这表明基础心电图表型在改善儿童 HCM 风险分层中的作用有限。
