The primary electrical disorders are a group of inherited cardiac ventricular arrhythmias that are a major cause of sudden cardiac death in young individuals. Inherited ventricular arrhythmias result from mutations in genes encoding cardiac ion channels or their modulatory subunits. Advances in genetic screening in the past three decades have led to the assembly of large patient cohorts with these disorders. Studies in these patients, as well as in the general population, have striven to define the prevalence of these inherited arrhythmias and the characteristics of patients with different genetic subtypes of the disease. In this Review, we provide a comprehensive update on the epidemiology of inherited ventricular arrhythmias, focusing on natural history, prevalence and patient demographics. In addition, we summarize the various founder populations (groups of individuals with a disease that is caused by a genetic defect inherited from a common ancestor) that have been identified for some of these disorders and which lead to increased prevalence in some geographical regions. To date, although numerous studies have markedly increased our understanding of the epidemiology of these disorders, demographic data, especially from non-Western countries, remain scarce. Furthermore, defining the true prevalence of these disorders remains challenging. International collaboration will undoubtedly accelerate the collection of demographic information and improve the accuracy of prevalence data.

原发性电障碍是一组遗传性心脏室性心律失常，它们是年轻人猝死的主要原因。遗传性室性心律失常是由编码心脏离子通道或其调节亚基的基因突变引起的。在过去的三十年中，基因筛查技术的进步导致了患有这些疾病的大型患者人群的聚集。这些患者以及普通人群的研究都在努力确定这些遗传性心律不齐的患病率以及具有该疾病不同遗传亚型的患者的特征。在这篇综述中，我们提供了关于遗传性室性心律失常流行病学的全面更新，重点是自然史，患病率和患者人口统计数据。此外，我们总结了已经为这些疾病中的某些疾病确定的各种创始人群（患有由共同祖先遗传的遗传缺陷引起的疾病的个体群体），这些人群导致某些地理区域的患病率增加。迄今为止，尽管大量研究显着增加了我们对这些疾病的流行病学的了解，但人口统计数据（尤其是来自非西方国家的人口统计数据）仍然匮乏。此外，确定这些疾病的真正患病率仍然具有挑战性。无疑，国际合作将加速人口统计信息的收集并提高流行率数据的准确性。