Normal electrocardiography at presentation masks SCN5A-related long QT syndrome: the importance of Holter monitoring in children with a family history of Brugada syndrome.

IF 0.8 4区医学 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

Cardiology in the Young Pub Date : 2026-09-30 DOI:10.1017/S1047951126124263

Hidenori Yamamoto, Kentaro Suzuki, Naoki Ohashi

引用次数: 0

Abstract

A 10-year-old girl who showed no abnormalities on resting or exercising stress electrocardiograms underwent Holter monitoring because of a family history of Brugada syndrome, which revealed marked QT prolongation during sleep. SCN5A-related inherited arrhythmia was suspected, and genetic testing confirmed the p.Glu1783Lys variant in SCN5A. Children with a family history of Brugada syndrome should undergo Holter monitoring proactively, suspecting the possibility of SCN5A-related inherited arrhythmia, even if 12-lead electrocardiograms show no abnormalities.

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出现时的正常心电图掩盖了scn5a相关的长QT综合征：Brugada综合征家族史患儿动态心电图监测的重要性

一名10岁女孩因Brugada综合征家族史，在静息或运动应激心电图上均无异常，接受了动态心电图监测，发现睡眠时QT间期明显延长。怀疑SCN5A相关的遗传性心律失常，基因检测证实了SCN5A的p.g ul1783lys变异。有Brugada综合征家族史的儿童，即使12导联心电图未显示异常，也应主动进行动态心电图监测，以怀疑scn5a相关遗传性心律失常的可能性。

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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来源期刊

Cardiology in the Young 医学-小儿科

CiteScore

1.70

自引率

10.00%

发文量

715

审稿时长

4-8 weeks

期刊介绍： Cardiology in the Young is devoted to cardiovascular issues affecting the young, and the older patient suffering the sequels of congenital heart disease, or other cardiac diseases acquired in childhood. The journal serves the interests of all professionals concerned with these topics. By design, the journal is international and multidisciplinary in its approach, and members of the editorial board take an active role in the its mission, helping to make it the essential journal in paediatric cardiology. All aspects of paediatric cardiology are covered within the journal. The content includes original articles, brief reports, editorials, reviews, and papers devoted to continuing professional development.