Exaggerated T-wave alternans in children with Angelman syndrome

Annals of the Child Neurology Society Pub Date : 2024-11-11 DOI:10.1002/cns3.20092

Eleonora Tamilia, Navaneethakrishna Makaram, Georgios Ntolkeras, Assia Chericoni, Sebastian Holst, Joerg Hipp, Alexander Rotenberg

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Abstract

Objective

We aimed to test whether T-wave alternans (TWA), which is a marker of susceptibility to ventricular fibrillation, is abnormal in children with Angelman syndrome (AS) compared with typically developing children (TDC), and whether it can be used as a biomarker of AS.

Materials and Methods

Using surface electrocardiogram (ECG), we calculated TWA in AS and compared it between AS and TDC (Wilcoxon rank sum test). We then performed logistic regression to test TWA ability to distinguish AS from TDC.

Results

We observed higher TWA in AS than TDC (44 vs. 33 uV, p = 0.009), while heart rate did not differ (p = 0.26), nor its variability (p = 0.72). TWA values enabled discrimination between AS and TDC (p = 0.0008) with accuracy of 81%, positive predictive value of 72%, and negative predictive value of 100%.

Interpretation

Our findings suggest that ECG in children with AS contains evidence of acquired cardiac abnormality via pathologically increased TWA.

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安杰尔曼综合征患儿的 T 波交替增快

目的探讨Angelman综合征（AS）患儿与正常发育儿童（TDC）相比，t波交替（TWA）作为心室颤动易感性指标是否存在异常，并探讨TWA是否可作为AS的生物标志物。材料与方法采用体表心电图（ECG）计算AS的TWA，并将AS与TDC进行比较（Wilcoxon秩和检验）。然后，我们进行了逻辑回归来测试TWA区分AS和TDC的能力。结果我们观察到AS患者的TWA高于TDC患者（44 vs. 33 uV, p = 0.009），而心率没有差异（p = 0.26），其变异性也没有差异（p = 0.72）。TWA值能够区分AS和TDC (p = 0.0008)，准确率为81%，阳性预测值为72%，阴性预测值为100%。我们的研究结果表明，AS儿童的心电图包含通过病理性TWA增加获得性心脏异常的证据。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Annals of the Child Neurology Society

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