Pharmacotherapy for inherited arrhythmia syndromes: mechanistic basis, clinical trial evidence and practical application

Expert Rev Cardiovasc Ther. 2015;13(7):769-82. doi: 10.1586/14779072.2015.1049156. Epub 2015 May 20.

Abstract

In the absence of structural heart disease, sudden cardiac death is frequently caused by inherited arrhythmia syndromes, such as long QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia. Managing these conditions often requires a combination of lifestyle modification, pharmacotherapy and less frequently, invasive therapy. Over the past decade, patient management has been greatly enhanced by tailored pharmacotherapy as a result of a deeper appreciation for arrhythmia mechanisms and supportive evidence from multicenter cohort studies. This article reviews current knowledge regarding drug therapy for inherited arrhythmias. Anti-arrhythmic mechanisms and available clinical evidence are highlighted while maintaining a practical perspective on patient management.

Keywords: Andersen Tawil syndrome; Brugada syndrome; catecholaminergic polymorphic ventricular tachycardia; channelopathy; early repolarization; idiopathic ventricular fibrillation; long QT syndrome; pharmacology; short QT syndrome; therapy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Action Potentials
  • Anti-Arrhythmia Agents / pharmacology
  • Anti-Arrhythmia Agents / therapeutic use*
  • Arrhythmias, Cardiac / drug therapy*
  • Arrhythmias, Cardiac / genetics
  • Arrhythmias, Cardiac / physiopathology
  • Humans
  • Ion Channels / drug effects
  • Ion Channels / physiology
  • Myocytes, Cardiac / drug effects
  • Myocytes, Cardiac / physiology

Substances

  • Anti-Arrhythmia Agents
  • Ion Channels