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(Circulation Research. 2004;94:140.)
© 2004 American Heart Association, Inc.

Reviews

Inherited Arrhythmogenic Diseases

The Complexity Beyond Monogenic Disorders

Silvia G. Priori

From Molecular Cardiology, IRCCS Fondazione Salvatore Maugeri, University of Pavia, Pavia, Italy.

Correspondence to Silvia G. Priori, MD, PhD, Molecular Cardiology, Maugeri Foundation, University of Pavia, Via Ferrata 8, 27100, Pavia, Italy. E-mail spriori{at}fsm.it

This Review is part of a thematic series on the Biology of Cardiac Arrhythmias, which includes the following articles:

Antiarrhythmic Drug Target Choices and Screening
Inherited Arrhythmogenic Diseases: The Complexity Beyond Monogenic Disorders
Genomics in Sudden Cardiac Death
Ion Channel Protein Processing
Regulation of Expression and Distribution of Ion Channels
Computational Insights: Chaos and Wave Theory
Gene Therapy and Cell Therapy of Cardiac Arrhythmias


This series is in honor of Harry A. Fozzard, 8th Editor of Circulation Research.
Gordon Tomaselli Editors

Twelve years after the identification of the molecular bases of the long-QT syndrome, it is now possible to express some considerations on the impact that genetic findings have had in the understanding of inherited arrhythmogenic diseases. Along with the excitement for the emerging data on genotype/phenotype correlation and for the development of the first recommendations for gene-specific management of patients, it is also important to acknowledge the unexpected complexity that has emerged. The focus of this article is to analyze the elusive aspects of the relationship between genetic defects and clinical manifestations and to propose some research directions that may provide the needed answers to move forward in the understanding of the genetics of heart rhythm abnormalities.

Key Words: arrhythmias • genetics • sudden cardiac death • ion channels • heart

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