Cardiomyocytes express a surprisingly large number of potassium channel types. The primary physiological functions of the currents conducted by these channels are to maintain the resting membrane potential and mediate action potential repolarization under basal conditions and in response to changes in the concentrations of intracellular sodium, calcium, and ATP/ADP. Here, we review the diversity and functional roles of cardiac potassium channels under normal conditions and how heritable mutations in the genes encoding these channels can lead to distinct arrhythmias. We briefly review atrial fibrillation and J-wave syndromes. For long and short QT syndromes, we describe their genetic basis, clinical manifestation, risk stratification, traditional and novel therapeutic approaches, as well as insights into disease mechanisms provided by animal and cellular models.

中文翻译：

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与心脏钾通道功能异常相关的遗传性心律失常。

心肌细胞表达数量惊人的钾通道类型。这些通道传导的电流的主要生理功能是在基础条件下维持静息膜电位并介导动作电位复极化，并响应细胞内钠、钙和 ATP/ADP 浓度的变化。在这里，我们回顾了正常条件下心脏钾通道的多样性和功能作用，以及编码这些通道的基因中的遗传突变如何导致不同的心律失常。我们简要回顾了心房颤动和 J 波综合征。对于长短 QT 综合征，我们描述了它们的遗传基础、临床表现、风险分层、传统和新的治疗方法、