Pathogenesis of familial inherited arrhythmias is being progressively clarified thanks to the insights provided by molecular biology and by functional studies. Transmembrane or intracellular ion channel mutations have been identified in genetically determined forms of polymorphic ventricular tachycardia and sudden death such as catecholaminergic ventricular tachycardia, long QT syndrome, and Brugada syndrome. The role of molecular abnormalities in the genesis of monomorphic idiopathic ventricular tachycardias is less well defined, mainly because of the lack of a Mendelian pattern of inheritance. Interestingly, the presence of somatic mutations has been suggested as the mechanism for monomorphic ventricular tachycardia originating from the right ventricular outflow tract. The future goals for the application of molecular genetics to the management of cardiac arrhythmias will be to apply molecular genetics for a better risk stratification of affected individuals and to aim for the identification of gene-specific treatment of idiopathic ventricular tachycardia.

Genetics of ventricular tachycardia.

Napolitano C;PRIORI, SILVIA GIULIANA
2002-01-01

Abstract

Pathogenesis of familial inherited arrhythmias is being progressively clarified thanks to the insights provided by molecular biology and by functional studies. Transmembrane or intracellular ion channel mutations have been identified in genetically determined forms of polymorphic ventricular tachycardia and sudden death such as catecholaminergic ventricular tachycardia, long QT syndrome, and Brugada syndrome. The role of molecular abnormalities in the genesis of monomorphic idiopathic ventricular tachycardias is less well defined, mainly because of the lack of a Mendelian pattern of inheritance. Interestingly, the presence of somatic mutations has been suggested as the mechanism for monomorphic ventricular tachycardia originating from the right ventricular outflow tract. The future goals for the application of molecular genetics to the management of cardiac arrhythmias will be to apply molecular genetics for a better risk stratification of affected individuals and to aim for the identification of gene-specific treatment of idiopathic ventricular tachycardia.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11571/438335
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