[座長] |
Charles Antzelevitch (Masonic Medical Research Laboratory, NY, USA) |
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大江 透 (心臓病センター榊原病院) |
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Genetic factors play a significant role in the pathogenesis of inherited cardiac arrhythmias. Modern molecular biological techniques have enabled identification and analysis of genes responsible for many of the inherited cardiac arrhythmia diseases. The most common inherited arrhythmic diseases predisposing to sudden death are long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia and short QT syndrome. It is now well recognized that there is a multiple genetic substrate for each clinical entity. More than ten different types of congenital long QT syndrome, and six in Brugada syndrome, two in catecholaminergic polymorphic ventricular tachycardia and three in short QT syndrome have been identified. Genotype-phenotype correlation studies have revealed that there are diverse clinical features depending on gene abnormality. It is also known that cardiac arrhythmias result from the interplay of genetic and environmental factors, suggesting that a more complex relationship exists between genotype and phenotype in these inherited cardiac arrhythmias.
The purpose of this symposium is to provide information on molecular mechanisms responsible for inherited cardiac arrhythmias and introduce recent findings regarding genotype-phenotype correlations.