Congenital long QT syndrome

Michael J. Ackerman; Anant Khositseth; David J. Tester; Peter J. Schwartz

doi:10.1007/978-1-84628-854-8_33

Congenital long QT syndrome

Michael J. Ackerman, Anant Khositseth, David J. Tester, Peter J. Schwartz

Cardiovascular Medicine

Research output: Chapter in Book/Report/Conference proceeding › Chapter

10 Scopus citations

Abstract

Once considered an extremely rare yet lethal arrhythmogenic peculiarity, congenital long QT syndrome (LQTS) is understood today as a primary cardiac arrhythmia syndrome (cardiac channelopathy) that is both far more common and, overall, much less lethal than previously recognized. Clinically, LQTS is often characterized by prolongation of the heart rate corrected QT interval (QTc) on a 12-lead surface electrocardiogram (ECG) and is associated with syncope, seizures, and sudden cardiac death due to ventricular arrhythmias (Torsade des pointes, TdP) usually following a precipitating event such as exertion, extreme emotion, or auditory stimulation. The molecular breakthroughs of the 1990s, led in large measure by the research laboratories of Drs. Mark Keating and Jeffrey Towbin in conjunction with LQTS registries containing meticulously phenotyped patients directed by Drs. Arthur Moss and Peter Schwartz, revealed the fundamental molecular underpinnings of LQTS- namely, defective cardiac channels.1

Original language	English (US)
Title of host publication	Electrical Diseases of the Heart
Subtitle of host publication	Genetics, Mechanisms, Treatment, Prevention
Publisher	Springer London
Pages	462-482
Number of pages	21
ISBN (Print)	9781846288531
DOIs	https://doi.org/10.1007/978-1-84628-854-8_33
State	Published - 2008

ASJC Scopus subject areas

General Medicine

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10.1007/978-1-84628-854-8_33

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abstract = "Once considered an extremely rare yet lethal arrhythmogenic peculiarity, congenital long QT syndrome (LQTS) is understood today as a primary cardiac arrhythmia syndrome (cardiac channelopathy) that is both far more common and, overall, much less lethal than previously recognized. Clinically, LQTS is often characterized by prolongation of the heart rate corrected QT interval (QTc) on a 12-lead surface electrocardiogram (ECG) and is associated with syncope, seizures, and sudden cardiac death due to ventricular arrhythmias (Torsade des pointes, TdP) usually following a precipitating event such as exertion, extreme emotion, or auditory stimulation. The molecular breakthroughs of the 1990s, led in large measure by the research laboratories of Drs. Mark Keating and Jeffrey Towbin in conjunction with LQTS registries containing meticulously phenotyped patients directed by Drs. Arthur Moss and Peter Schwartz, revealed the fundamental molecular underpinnings of LQTS- namely, defective cardiac channels.1",

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AU - Ackerman, Michael J.

AU - Khositseth, Anant

AU - Tester, David J.

AU - Schwartz, Peter J.

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N2 - Once considered an extremely rare yet lethal arrhythmogenic peculiarity, congenital long QT syndrome (LQTS) is understood today as a primary cardiac arrhythmia syndrome (cardiac channelopathy) that is both far more common and, overall, much less lethal than previously recognized. Clinically, LQTS is often characterized by prolongation of the heart rate corrected QT interval (QTc) on a 12-lead surface electrocardiogram (ECG) and is associated with syncope, seizures, and sudden cardiac death due to ventricular arrhythmias (Torsade des pointes, TdP) usually following a precipitating event such as exertion, extreme emotion, or auditory stimulation. The molecular breakthroughs of the 1990s, led in large measure by the research laboratories of Drs. Mark Keating and Jeffrey Towbin in conjunction with LQTS registries containing meticulously phenotyped patients directed by Drs. Arthur Moss and Peter Schwartz, revealed the fundamental molecular underpinnings of LQTS- namely, defective cardiac channels.1

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Congenital long QT syndrome

Abstract

ASJC Scopus subject areas

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