Curr Probl Cardiol. 2012 Aug;37(8):317-62. doi: 10.1016/j.cpcardiol.2012.03.004.
Repolarization syndromes, including early repolarization, Brugada, and short and long QT, have been implicated increasingly as causes of sudden cardiac death (SCD) despite no obvious mechanical cardiac abnormalities. So-called idiopathic ventricular fibrillation is now often reassigned to one of the aforementioned entities. Underlying causes are diverse; genetic mutation has been proven in many but not all cases. Although high-risk individuals generally can be identified, most of the potential victim pool is still unknown and cannot be discovered at this time. Awareness of these entities' existence, knowledge of family history, and 12-lead electrocardiography are the initial steps toward preventing SCD in this population. Underlying mechanisms for ventricular tachycardia/fibrillation in such individuals include phase 2 reentry, early after depolarization, and vortex reentry. For the time-being, although most forms of long QT syndrome can be treated with β-blockers, an implantable cardioverter-defibrillator remains the only definitive therapy for the prevention of arrhythmic death among high-risk populations.
复极综合征,包括早期复极、Brugada 综合征以及短 QT 和长 QT 综合征,尽管心脏无明显机械性异常,但其与心脏性猝死(SCD)的关系越来越受到关注。所谓的特发性心室颤动现在通常被重新归类为上述一种综合征。潜在病因多种多样;虽然已在许多但不是所有病例中证实存在基因突变。尽管一般可以识别高危个体,但大部分潜在的受害者群体仍未知,目前尚无法发现。了解这些实体的存在、家族病史以及 12 导联心电图是预防该人群 SCD 的初始步骤。在这些个体中,室性心动过速/颤动的潜在机制包括 2 相折返、早期后除极和涡流折返。目前,尽管大多数形式的长 QT 综合征可以用β受体阻滞剂治疗,但植入式心脏复律除颤器仍然是预防高危人群心律失常性死亡的唯一明确治疗方法。