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Inherited arrhythmias: a National Heart, Lung, and Blood Institute and Office of Rare Diseases workshop consensus report about the diagnosis, phenotyping, molecular mechanisms, and therapeutic approaches for primary cardiomyopathies of gene mutations affe

Abstract：

:The National Heart, Lung, and Blood Institute and Office of Rare Diseases at the National Institutes of Health organized a workshop (September 14 to 15, 2006, in Bethesda, Md) to advise on new research directions needed for improved identification and treatment of rare inherited arrhythmias. These included the following: (1) Na+ channelopathies; (2) arrhythmias due to K+ channel mutations; and (3) arrhythmias due to other inherited arrhythmogenic mechanisms. Another major goal was to provide recommendations to support, enable, or facilitate research to improve future diagnosis and management of inherited arrhythmias. Classifications of electric heart diseases have proved to be exceedingly complex and in many respects contradictory. A new contemporary and rigorous classification of arrhythmogenic cardiomyopathies is proposed. This consensus report provides an important framework and overview to this increasingly heterogeneous group of primary cardiac membrane channel diseases. Of particular note, the present classification scheme recognizes the rapid evolution of molecular biology and novel therapeutic approaches in cardiology, as well as the introduction of many recently described diseases, and is unique in that it incorporates ion channelopathies as a primary cardiomyopathy in consensus with a recent American Heart Association Scientific Statement.

journal_name

Circulation

journal_title

Circulation

authors

Lehnart SE,Ackerman MJ,Benson DW Jr,Brugada R,Clancy CE,Donahue JK,George AL Jr,Grant AO,Groft SC,January CT,Lathrop DA,Lederer WJ,Makielski JC,Mohler PJ,Moss A,Nerbonne JM,Olson TM,Przywara DA,Towbin JA,Wang LH,M

doi

10.1161/CIRCULATIONAHA.107.711689

subject

Has Abstract

pub_date

2007-11-13 00:00:00

pages

2325-45

issue

eissn

0009-7322

issn

1524-4539

pii

116/20/2325

journal_volume

116

pub_type

杂志文章

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Inherited arrhythmias: a National Heart, Lung, and Blood Institute and Office of Rare Diseases workshop consensus report about the diagnosis, phenotyping, molecular mechanisms, and therapeutic approaches for primary cardiomyopathies of gene mutations affe