The usual suspects in sudden cardiac death of the young: a focus on inherited arrhythmogenic diseases

Expert Rev Cardiovasc Ther. 2014 Apr;12(4):499-519. doi: 10.1586/14779072.2014.894884.

Abstract

Up to 14,500 young individuals die suddenly every year in Europe of cardiac pathologies. The majority of these tragic events are related to a group of genetic defects that predispose the development of malignant arrhythmias (inherited arrhythmogenic diseases [IADs]). IADs include both cardiomyopathies (hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy) and channelopathies (long QT syndrome, short QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia). Every time an IAD is identified in a patient, other individuals in his/her family may be at risk of cardiac events. However; if a timely diagnosis is made, simple preventative measures may be applied. Genetic studies play a pivotal role in the diagnosis of IADs and may help in the management of patients and their relatives.

Publication types

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

MeSH terms

  • Arrhythmias, Cardiac / complications
  • Arrhythmias, Cardiac / diagnosis*
  • Arrhythmias, Cardiac / prevention & control
  • Brugada Syndrome / diagnosis*
  • Brugada Syndrome / genetics
  • Brugada Syndrome / prevention & control
  • Cardiomyopathies / diagnosis*
  • Cardiomyopathies / pathology
  • Cardiomyopathies / physiopathology
  • Cardiomyopathies / prevention & control
  • Death, Sudden, Cardiac / etiology*
  • Death, Sudden, Cardiac / prevention & control
  • Diagnosis, Differential
  • Disease Susceptibility
  • Humans
  • Tachycardia, Ventricular / complications
  • Tachycardia, Ventricular / diagnosis*
  • Tachycardia, Ventricular / genetics
  • Tachycardia, Ventricular / prevention & control

Supplementary concepts

  • Polymorphic catecholergic ventricular tachycardia