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

Stephan E. Lehnart, Michael J. Ackerman, D. Woodrow Benson, Ramon Brugada, Colleen E. Clancy, J. Kevin Donahue, Alfred L. George, Augustus O. Grant, Stephen C. Groft, Craig T. January, David A. Lathrop, W. Jonathan Lederer, Jonathan C. Makielski, Peter J. Mohler, Arthur Moss, Jeanne M. Nerbonne, Timothy M. Olson, Dennis A. Przywara, Jeffrey Towbin, Lan Hsiang Wang & 1 others Andrew R. Marks

Research output: Contribution to journalArticle

195 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)2325-2345
Number of pages21
JournalCirculation
Volume116
Issue number20
DOIs
StatePublished - Nov 1 2007

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Consensus Development Conferences
National Heart, Lung, and Blood Institute (U.S.)
Rare Diseases
Cardiomyopathies
Ion Channels
Cardiac Arrhythmias
Channelopathies
Mutation
Genes
National Institutes of Health (U.S.)
Therapeutics
Cardiology
Research
Molecular Biology
Heart Diseases
Ions
Education

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

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. / Lehnart, Stephan E.; Ackerman, Michael J.; Benson, D. Woodrow; Brugada, Ramon; Clancy, Colleen E.; Donahue, J. Kevin; George, Alfred L.; Grant, Augustus O.; Groft, Stephen C.; January, Craig T.; Lathrop, David A.; Lederer, W. Jonathan; Makielski, Jonathan C.; Mohler, Peter J.; Moss, Arthur; Nerbonne, Jeanne M.; Olson, Timothy M.; Przywara, Dennis A.; Towbin, Jeffrey; Wang, Lan Hsiang; Marks, Andrew R.

In: Circulation, Vol. 116, No. 20, 01.11.2007, p. 2325-2345.

Research output: Contribution to journalArticle

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, J, Wang, LH & Marks, AR 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, vol. 116, no. 20, pp. 2325-2345. https://doi.org/10.1161/CIRCULATIONAHA.107.711689
Lehnart, Stephan E. ; Ackerman, Michael J. ; Benson, D. Woodrow ; Brugada, Ramon ; Clancy, Colleen E. ; Donahue, J. Kevin ; George, Alfred L. ; Grant, Augustus O. ; Groft, Stephen C. ; January, Craig T. ; Lathrop, David A. ; Lederer, W. Jonathan ; Makielski, Jonathan C. ; Mohler, Peter J. ; Moss, Arthur ; Nerbonne, Jeanne M. ; Olson, Timothy M. ; Przywara, Dennis A. ; Towbin, Jeffrey ; Wang, Lan Hsiang ; Marks, Andrew R. / 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. In: Circulation. 2007 ; Vol. 116, No. 20. pp. 2325-2345.
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AU - Brugada, Ramon

AU - Clancy, Colleen E.

AU - Donahue, J. Kevin

AU - George, Alfred L.

AU - Grant, Augustus O.

AU - Groft, Stephen C.

AU - January, Craig T.

AU - Lathrop, David A.

AU - Lederer, W. Jonathan

AU - Makielski, Jonathan C.

AU - Mohler, Peter J.

AU - Moss, Arthur

AU - Nerbonne, Jeanne M.

AU - Olson, Timothy M.

AU - Przywara, Dennis A.

AU - Towbin, Jeffrey

AU - Wang, Lan Hsiang

AU - Marks, Andrew R.

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N2 - 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.

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