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Congenital Long QT-Syndrome

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Clinical Cardiogenetics

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Abstract

The congenital long QT syndrome (LQTS) is considered to be one of the hereditary cardiac arrhythmia syndromes, nowadays also known as cardiac channelopathies. The syndrome is characterized by prolongation of the heart rate corrected QT-interval (QTc) on the 12-lead electrocardiogram (ECG). In affected family members, it is associated with recurrent syncope, seizures, and sudden cardiac death due to ventricular arrhythmias (Torsade des Pointes (TdP) and ventricular fibrillation), which typically follow a precipitating event such as exertion, extreme emotion, swimming and diving, or auditory stimulation.

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Authors and Affiliations

  1. Department of cardiology, TweeSteden Hospital Tilburg and department of cardiology, Heart and Lung Center Utrecht, University Medical Centre Utrecht, Utrecht, The Netherlands

    Hubert F. Baars

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  1. Hubert F. Baars
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  2. Jeroen F. van der Heijden
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Correspondence to Hubert F. Baars .

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  1. University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, 3508 GA, Netherlands

    H.F. Baars

  2. University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, 3508 GA, Netherlands

    P.A.F.M. Doevendans

  3. University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, 3508 GA, Netherlands

    J.J. van der Smagt

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© 2011 Springer-Verlag London Limited

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Baars, H.F., van der Heijden, J.F. (2011). Congenital Long QT-Syndrome. In: Baars, H., Doevendans, P., van der Smagt, J. (eds) Clinical Cardiogenetics. Springer, London. https://doi.org/10.1007/978-1-84996-471-5_9

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  • DOI: https://doi.org/10.1007/978-1-84996-471-5_9

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