Repolarization syndromes

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.

Publication types

  • Review

MeSH terms

  • Arrhythmias, Cardiac / complications
  • Arrhythmias, Cardiac / diagnosis*
  • Arrhythmias, Cardiac / genetics
  • Arrhythmias, Cardiac / therapy
  • Brugada Syndrome / diagnosis
  • Brugada Syndrome / genetics
  • Brugada Syndrome / therapy
  • Death, Sudden, Cardiac / etiology
  • Death, Sudden, Cardiac / prevention & control
  • Electrocardiography
  • Genetic Predisposition to Disease
  • Humans
  • Long QT Syndrome / diagnosis
  • Long QT Syndrome / genetics
  • Long QT Syndrome / therapy
  • Mutation