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Long and Short QT Syndromes

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Channelopathies in Heart Disease

Part of the book series: Cardiac and Vascular Biology ((Abbreviated title: Card. vasc. biol.,volume 6))

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Abstract

The long and short QT syndromes are genetically transmitted arrhythmogenic diseases characterized by an abnormal QTc on the basal ECG and by an increased risk of life-threatening arrhythmias. While in the long QT syndrome well-established diagnostic criteria are available as well as effective treatments, in the short QT syndrome, much less is known in terms of diagnosis, risk stratification and pharmacological treatment. In this chapter we discuss for each syndrome current knowledge on their genetic basis, clinical presentation, diagnosis, risk stratification and therapy. Furthermore, multisystem disorders associated with a prolongation of the QT, such as the Jervell and Lange-Nielsen syndrome, the Timothy syndrome, the ankyrin-B syndrome and the Andersen-Tawil syndrome, are described. Finally, specific subtypes of the long QT syndrome, characterized by high malignancy and frequent failure of available therapies, such as calmodulin-related LQTS and the triadin knockout syndrome, are also reviewed.

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Authors and Affiliations

  1. IRCCS Istituto Auxologico Italiano, Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Milan, Italy

    Lia Crotti, Maria-Christina Kotta & Silvia Castelletti

  2. Department of Cardiovascular, Neural and Metabolic Sciences, San Luca Hospital, IRCCS Istituto Auxologico Italiano, Milan, Italy

    Lia Crotti

  3. Department of Medicine and Surgery, University of Milano-Bicocca, Milan, Italy

    Lia Crotti

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  3. Silvia Castelletti
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Correspondence to Lia Crotti .

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Editors and Affiliations

  1. Department of Cardiology and HCR (Heidelberg Center for Heart Rhythm Disorders), University Hospital Heidelberg, Heidelberg, Germany

    Dierk Thomas

  2. Dept. of Clinical & Experimental Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

    Carol Ann Remme

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LC declares that she has no conflict of interest. MCK declares that she has no conflict of interest. SC declares that she has no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.

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Crotti, L., Kotta, MC., Castelletti, S. (2018). Long and Short QT Syndromes. In: Thomas, D., Remme, C. (eds) Channelopathies in Heart Disease . Cardiac and Vascular Biology, vol 6. Springer, Cham. https://doi.org/10.1007/978-3-319-77812-9_7

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