Congenital long QT syndrome

David J. Tester, Peter J. Schwartz, Michael John Ackerman

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

With an incidence as high as 1 in 2,000-2,500 live births, long QT syndrome (LQTS) is often characterized clinically 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 usually following a precipitating event such as exertion, extreme emotion, or auditory stimulation. The phenotypic expression of LQTS varies profoundly from asymptomatic longevity to premature sudden cardiac death despite medical therapy. Therefore the clinical/genetic diagnostic evaluation and risk-stratification are highly important issues in the clinical management of LQTS. This chapter will review the historical background, epidemiology and prevalence, molecular genetics, and clinical presentations of LQTS, explore unique genotype - phenotype relationships that help define the various forms of the disorder, and provide a detailed outline for the diagnostic evaluation and clinical management of LQTS patients including current treatment strategies and recommendations.

Original languageEnglish (US)
Title of host publicationElectrical Diseases of the Heart
PublisherSpringer-Verlag London Ltd
Pages439-468
Number of pages30
Volume1
ISBN (Print)9781447148814, 1447148800, 9781447148807
DOIs
StatePublished - Mar 1 2013

Fingerprint

Long QT Syndrome
Sudden Cardiac Death
Acoustic Stimulation
Syncope
Live Birth
Cardiac Arrhythmias
Molecular Biology
Electrocardiography
Emotions
Epidemiology
Seizures
Heart Rate
Genotype
Phenotype
Incidence
Therapeutics

Keywords

  • Electrocardiogram
  • Genetic testing
  • Genetics
  • Ion channels
  • Long QT syndrome
  • QT interval
  • Sudden death

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Tester, D. J., Schwartz, P. J., & Ackerman, M. J. (2013). Congenital long QT syndrome. In Electrical Diseases of the Heart (Vol. 1, pp. 439-468). Springer-Verlag London Ltd. https://doi.org/10.1007/978-1-4471-4881-4_27

Congenital long QT syndrome. / Tester, David J.; Schwartz, Peter J.; Ackerman, Michael John.

Electrical Diseases of the Heart. Vol. 1 Springer-Verlag London Ltd, 2013. p. 439-468.

Research output: Chapter in Book/Report/Conference proceedingChapter

Tester, DJ, Schwartz, PJ & Ackerman, MJ 2013, Congenital long QT syndrome. in Electrical Diseases of the Heart. vol. 1, Springer-Verlag London Ltd, pp. 439-468. https://doi.org/10.1007/978-1-4471-4881-4_27
Tester DJ, Schwartz PJ, Ackerman MJ. Congenital long QT syndrome. In Electrical Diseases of the Heart. Vol. 1. Springer-Verlag London Ltd. 2013. p. 439-468 https://doi.org/10.1007/978-1-4471-4881-4_27
Tester, David J. ; Schwartz, Peter J. ; Ackerman, Michael John. / Congenital long QT syndrome. Electrical Diseases of the Heart. Vol. 1 Springer-Verlag London Ltd, 2013. pp. 439-468
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