Congenital long QT syndrome

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

Research output: Chapter in Book/Report/Conference proceedingChapter

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 languageEnglish
Title of host publicationElectrical Diseases of the Heart: Genetics, Mechanisms, Treatment, Prevention
PublisherSpringer London
Pages462-482
Number of pages21
ISBN (Print)9781846288531
DOIs
Publication statusPublished - 2008

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Ackerman, M. J., Khositseth, A., Tester, D. J., & Schwartz, P. J. (2008). Congenital long QT syndrome. In Electrical Diseases of the Heart: Genetics, Mechanisms, Treatment, Prevention (pp. 462-482). Springer London. https://doi.org/10.1007/978-1-84628-854-8_33