Molecular Genetics of Cardiac Electrophysiology

by Berul, Charles I.; Towbin, Jeffrey A., M.D.

• ISBN13: 9780792378297
• ISBN10: 0792378296
• Format: Hardcover
• Copyright: 2000-04-30
• Publisher: Kluwer Academic Pub

Summary

The field of molecular genetics has rapidly expanded and is becoming increasingly important in the area of cardiac electrophysiology. The genetic basis of cardiac conduction disturbances and arrhythmogenesis is now recognized as a critical factor in disease pathology. Inherited structural and electrical heart diseases are being elucidated using molecular biological approaches, with differentiation of gene-specific diagnosis, prognostication, and therapeutics. Unique genetically-engineered animal models of hereditary arrhythmias and heart disease are being developed and utilized for detailed characterization of the cardiac conduction system. The goal of this book is to provide the adult and pediatric cardiologist, electrophysiologist, geneticist, internist, pediatrician, and trainee with a comprehensive review and update of this new field involving the molecular genetics of cardiac electrophysiology.

Table of Contents

Overview: Genetics of Cardiac Electrophysiology	1	(2)
Jeffrey A. Towbin
Charles I. Berul
Section I. Experimental Investigation of Inherited Electrophysiological Disorders
Single-Cell Electrophysiology & Ion Channelopathies	3	(20)
Coeli Lopes
Steve A. N. Goldstein
Michael Apkon
Animal Models of Inherited Electrophysiologic Diseases	23	(22)
Amit Rakhit
Charles I. Berul
Optical Mapping of Arrhythmias	45	(16)
Gregory E. Morley
Dhananjay Vaidya
Jose Jalife
Connexins and Conduction	61	(20)
Deborah Lerner
Jeffrey Saffitz
Section II. Hereditary Arrhythmias
Hereditary Supraventricular Tachycardias	81	(12)
Patrick Y. Jay
Charles I. Berul
Familial Atrial Fibrillation	93	(8)
Ramon Brugada
Robert Roberts
The Molecular Cardiology of Heart Block	101	(18)
Robert M. Hamilton
Section III. Ion Channel Mutations with Clinical Electrophysiologic Consequences
Congenital Long QT Syndromes	119	(18)
G. Michael Vincent
Katherine Timothy
Molecular Genetics of the Acquired Long QT Syndrome	137	(10)
Dan Roden
Brugada Syndrome Genetics	147	(34)
Jeffrey A. Towbin
Matteo Vatta
Koonalee Nademanee
Ramon Brugada
Josep Brugada
Charles Antzelevith
Pedro Brugada
Section IV. Inherited Cardiomyopathies with Arrhythmias
Familial Hypertrophic Cardiomyopathy Genetics	181	(14)
Laura M. Bevilacqua
Charles I. Berul
Familial Dilated Cardiomyopathy	195	(24)
Jeffrey A. Towbin
Neil E. Bowles
Glycogen Storage Diseases	219	(20)
Mira Irons
Ellen Roy Elias
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy	239	(12)
Frank I. Marcus
Peter Ott
Cardiac Disease in Duchenne & Becker Muscular Dystrophies: The Dystrophinopathies	251	(16)
Leslie B. Smoot
Gerald Cox
Myotonic Muscular Dystrophy Genetics and Cardiac Sequelae	267	(20)
Sita Reddy
Charles I. Berul
Section V. Inherited Structural Heart Diseases Associated with Arrhythmias
Genetic Causes of Atrial Septal Defects	287	(10)
Kristen Patton
Christine E. Seidman
Holt-Oram Syndrome and the TBX5 Transcription Factor in Cardiogenesis	297	(20)
Cathy J. Hatcher
Craig T. Basson
Inherited Structural Heart Diseases Associated With Arrhythmias: Defects in Laterality	317	(12)
Baruch S. Ticho
Richard Van Praagh
Marfan Syndrome and Related Disorders of Fibrillin	329	(26)
Ronald V. Lacro
The Molecular Genetics of Conotruncal Defects	355	(20)
Elizabeth Goldmuntz
Index	375