Despite significant advances in the prevention of cardiovascular diseases, sudden cardiac death (SCD) persists as a major public health problem. Among young and apparently healthy individuals, Long-QT syndrome (LQTS) represents a leading progenitor of SCD owing to fatal ventricular arrhythmia. Scientific understanding of this association has grown in recent years, and the mortality rate after LQTS diagnosis has significantly decreased. However, despite medical treatment advances, life-threatening ventricular arrhythmias still occur. Until now, no research has established the degree to which this inherited condition arises from an underlying arrhythmogenic electroanatomical substrate. Here, we present direct evidence showing that LQTS patients who survive spontaneous malignant arrhythmias harbor structural electrophysiological abnormalities localized in the epicardium of the right ventricle. We further show that the elimination of these abnormalities by means of catheter ablation successfully suppresses malignant arrhythmias, offering a new approach for the effective treatment of LQTS patients.

中文翻译：

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长QT综合征猝死的基质位于心外膜

尽管在预防心血管疾病方面取得了重大进展，但心源性猝死 (SCD) 仍然是一个主要的公共卫生问题。在年轻且表面上健康的个体中，长 QT 综合征 (LQTS) 是致命性室性心律失常导致 SCD 的主要祖先。近年来对这种关联的科学认识有所增加，LQTS 诊断后的死亡率显着下降。然而，尽管医学治疗有所进步，但仍会发生危及生命的室性心律失常。到目前为止，还没有研究确定这种遗传性疾病在多大程度上是由潜在的致心律失常电解剖底物引起的。这里，我们提供的直接证据表明，自发性恶性心律失常幸存的 LQTS 患者具有位于右心室心外膜的结构电生理异常。我们进一步表明，通过导管消融消除这些异常成功地抑制了恶性心律失常，为 LQTS 患者的有效治疗提供了一种新方法。