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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 affecting ion channel function.

Publication ,  Journal Article
Lehnart, SE; Ackerman, MJ; Benson, DW; Brugada, R; Clancy, CE; Donahue, JK; George, AL; Grant, AO; Groft, SC; January, CT; Lathrop, DA ...
Published in: Circulation
November 13, 2007

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.

Duke Scholars

Published In

Circulation

DOI

EISSN

1524-4539

Publication Date

November 13, 2007

Volume

116

Issue

20

Start / End Page

2325 / 2345

Location

United States

Related Subject Headings

  • United States
  • Phenotype
  • National Heart, Lung, and Blood Institute (U.S.)
  • Mutation
  • Long QT Syndrome
  • Humans
  • Channelopathies
  • Cardiovascular System & Hematology
  • Cardiomyopathies
  • Arrhythmias, Cardiac
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Lehnart, S. E., Ackerman, M. J., Benson, D. W., Brugada, R., Clancy, C. E., Donahue, J. K., … Marks, A. R. (2007). 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 affecting ion channel function. Circulation, 116(20), 2325–2345. https://doi.org/10.1161/CIRCULATIONAHA.107.711689
Lehnart, Stephan E., Michael J. Ackerman, D Woodrow Benson, Ramon Brugada, Colleen E. Clancy, J Kevin Donahue, Alfred L. George, et al. “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 affecting ion channel function.Circulation 116, no. 20 (November 13, 2007): 2325–45. https://doi.org/10.1161/CIRCULATIONAHA.107.711689.
Lehnart SE, Ackerman MJ, Benson DW, Brugada R, Clancy CE, Donahue JK, George AL, 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 L-H, Marks AR. 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 affecting ion channel function. Circulation. 2007 Nov 13;116(20):2325–2345.

Published In

Circulation

DOI

EISSN

1524-4539

Publication Date

November 13, 2007

Volume

116

Issue

20

Start / End Page

2325 / 2345

Location

United States

Related Subject Headings

  • United States
  • Phenotype
  • National Heart, Lung, and Blood Institute (U.S.)
  • Mutation
  • Long QT Syndrome
  • Humans
  • Channelopathies
  • Cardiovascular System & Hematology
  • Cardiomyopathies
  • Arrhythmias, Cardiac