Long QT syndrome 3

Common Name(s)

Long QT syndrome 3

Congenital long QT syndrome is electrocardiographically characterized by a prolonged QT interval and polymorphic ventricular arrhythmias (torsade de pointes). These cardiac arrhythmias may result in recurrent syncope, seizure, or sudden death ({4:Jongbloed et al., 1999}). For a discussion of genetic heterogeneity of long QT syndrome, see LQT1 ({192500}).
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Long QT syndrome 3" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Long QT syndrome 3" returned 24 free, full-text research articles on human participants. First 3 results:

Gene-Specific Therapy With Mexiletine Reduces Arrhythmic Events in Patients With Long QT Syndrome Type 3.
 

Author(s): Andrea Mazzanti, Riccardo Maragna, Alessandro Faragli, Nicola Monteforte, Raffaella Bloise, Mirella Memmi, Valeria Novelli, Paola Baiardi, Vincenzo Bagnardi, Susan P Etheridge, Carlo Napolitano, Silvia G Priori

Journal: J. Am. Coll. Cardiol.. 2016 Mar;67(9):1053-8.

 

Long QT syndrome type 3 (LQT3) is a lethal disease caused by gain-of-function mutations in the SCN5A gene, coding for the alpha-subunit of the sodium channel NaV1.5. Mexiletine is used to block late sodium current and to shorten QT interval in LQT3 patients.

Last Updated: 4 Mar 2016

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SCN5A mutations associated with overlap phenotype of long QT syndrome type 3 and Brugada syndrome.
 

Author(s): Haruaki Nakaya

Journal: Circ. J.. 2014 ;78(5):1061-2.

 

Last Updated: 28 Apr 2014

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The disease-specific phenotype in cardiomyocytes derived from induced pluripotent stem cells of two long QT syndrome type 3 patients.
 

Author(s): Azra Fatima, Shao Kaifeng, Sven Dittmann, Guoxing Xu, Manoj K Gupta, Matthias Linke, Ulrich Zechner, Filomain Nguemo, Hendrik Milting, Martin Farr, Jürgen Hescheler, Tomo Sarić

Journal:

 

Long QT syndromes (LQTS) are heritable diseases characterized by prolongation of the QT interval on an electrocardiogram, which often leads to syncope and sudden cardiac death. Here we report the generation of induced pluripotent stems (iPS) cells from two patients with LQTS type ...

Last Updated: 18 Dec 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Long QT syndrome 3" returned 0 free, full-text review articles on human participants.

 
 
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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Worm Study: Modifier Genes in Sudden Cardiac Death
 

Status: Recruiting

Condition Summary: Brugada Syndrome; Long QT Syndrome 3

 

Last Updated: 13 May 2015

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Efficacy Study of Sodium Channel Blocker in LQT3 Patients
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 30 Dec 2015

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Long Term Prophylactic Therapy of Congenital Long QT Syndrome Type III (LQT3) With Ranolazine
 

Status: Recruiting

Condition Summary: Long QT Syndrome Type 3

 

Last Updated: 25 Mar 2015

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