Concealed arrhythmogenic syndromes: the hidden substrate of idiopathic ventricular fibrillation?

Cardiovasc Res. 2001 May;50(2):218-23. doi: 10.1016/s0008-6363(01)00224-3.


In approximately 6--10% of survivors of cardiac arrest no cardiac abnormality can be identified despite extensive clinical evaluation. Autopsy data confirm that in a similar percentage of victims of sudden death no structural heart disease can be identified at post mortem evaluation. Occurrence of cardiac arrest in the absence of a substrate is defined 'idiopathic ventricular fibrillation' thus admitting that the cause for the arrhythmic event has remained unknown. We present data supporting the hypothesis that incompletely penetrant genetic defects may underlie at least some of these unexplained deaths.

Publication types

  • Review

MeSH terms

  • Death, Sudden, Cardiac / etiology*
  • Death, Sudden, Cardiac / pathology
  • Electrocardiography
  • Humans
  • Long QT Syndrome / complications
  • Pedigree
  • Ventricular Fibrillation / genetics*