Boussy Tim, Paparella Gaetano, de Asmundis Carlo, Sarkozy Andrea, Chierchia Gian Battista, Brugada Josep, Brugada Ramon, Brugada Pedro
Heart Rhythm Management Institute, University Hospital Brussels, Belgium, Laarbeeklaan 101 - 1090 Brussels, Belgium.
Cardiol Clin. 2008 Aug;26(3):335-53, v. doi: 10.1016/j.ccl.2008.03.005.
Sudden cardiac death caused by malignant ventricular arrhythmias is the most important cause of death in the industrialized world. Most of these lethal arrhythmias occur in the setting of ischemic heart disease. A significant number of sudden deaths, especially in young individuals, are caused by inherited ventricular arrhythmic disorders, however. Genetically induced ventricular arrhythmias can be divided in two subgroups: the primary electrical disorders or channelopathies, and the secondary arrhythmogenic cardiomyopathies. This article focuses on the genetic background of these electrical disorders and the current knowledge of genotype-phenotype interactions.
由恶性室性心律失常导致的心脏性猝死是工业化国家最重要的死亡原因。这些致命性心律失常大多发生在缺血性心脏病的背景下。然而，相当数量的猝死，尤其是在年轻人中，是由遗传性室性心律失常疾病引起的。基因诱导的室性心律失常可分为两个亚组：原发性电紊乱或离子通道病，以及继发性致心律失常性心肌病。本文重点关注这些电紊乱的遗传背景以及目前关于基因型-表型相互作用的知识。
