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Cardiovascular Research 2001 50(2):218-223; doi:10.1016/S0008-6363(01)00224-3
© 2001 by European Society of Cardiology
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Copyright © 2001, European Society of Cardiology

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

Silvia G Prioria,b,*, Carlo Napolitanoa and Massimiliano Grillob

aMolecular Cardiology, Fondazione Salvatore Maugeri, Via Ferrata 8, 27100 Pavia, Italy
bDepartment of Cardiology, University of Pavia, Pavia, Italy

* Corresponding author. Present address: Molecular Cardiology, Fondazione Salvatore Maugeri, Via Ferrata 8, 27100 Pavia, Italy. Tel.: +39-0382-592-051; fax: +39-0382-592-094 spriori{at}fsm.it

In ~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.

KEYWORDS Arrhythmia (mechanisms); Long QT syndrome; Ventricular arrhythmias; Sudden death


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