14th International Congress
THE "NEW FRONTIERS"
OF ARRHYTHMIAS 2000

Jan. 29 - Feb. 5, 2000
Marilleva, Trento, Italy

RT-193

Ion channel diseases and sudden cardiac death

Silvia G. Priori, Marina Cerrone.
Molecular Cardiology Laboratories, Fondazione Salvatore Maugeri IRCCS, Pavia, Policlinico S.Matteo IRCCS, Pavia, Italy

Introduction

Sudden cardiac death accounts for approximately 300000 deaths every year. The vast majority of these deaths occurs as a consequence of coronary artery disease or of other structural defects such as cardiomyopathies. In a minority of cases, sudded cardiac death is the first clinical manifestation in apparently healthy individuals and even after autopsy its cause may remain unknown. The name idiopathic ventricular fibrillation¹ is frequently used to refer to these unexplained cases. Recently some light has been shed on the possible substrate of idiopathic sudden cardiac death through the identification of inherited abnormalities of cardiac ion channels that may predispose the heart to an “electrical” failure^2-4.

In the following pages we will provide a short review on the principles of “cardiac channellopaties” and their genetic substrate. The future implications of these novel findings will be discussed as they may represent the missing link between inherited and acquired premature sudden death.