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.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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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 27 free, full-text research articles on human participants. First 3 results:

Clinical Aspects of Type 3 Long-QT Syndrome: An International Multicenter Study.
 

Author(s): Arthur A M Wilde, Arthur J Moss, Elizabeth S Kaufman, Wataru Shimizu, Derick R Peterson, Jesaia Benhorin, Coeli Lopes, Jeffrey A Towbin, Carla Spazzolini, Lia Crotti, Wojciech Zareba, Ilan Goldenberg, Jørgen K Kanters, Jennifer L Robinson, Ming Qi, Nynke Hofman, David J Tester, Connie R Bezzina, Marielle Alders, Takeshi Aiba, Shiro Kamakura, Yoshihiro Miyamoto, Mark L Andrews, Scott McNitt, Bronislava Polonsky, Peter J Schwartz, Michael J Ackerman

Journal: Circulation. 2016 Sep;134(12):872-82.

 

Risk stratification in patients with type 3 long-QT syndrome (LQT3) by clinical and genetic characteristics and effectiveness of β-blocker therapy has not been studied previously in a large LQT3 population.

Last Updated: 20 Sep 2016

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Genetic Modifiers for the Long-QT Syndrome: How Important Is the Role of Variants in the 3' Untranslated Region of KCNQ1?
 

Author(s): Lia Crotti, Annukka M Lahtinen, Carla Spazzolini, Elisa Mastantuono, Maria Cristina Monti, Caterina Morassutto, Gianfranco Parati, Marshall Heradien, Althea Goosen, Peter Lichtner, Thomas Meitinger, Paul A Brink, Kimmo Kontula, Heikki Swan, Peter J Schwartz

Journal: Circ Cardiovasc Genet. 2016 Aug;9(4):330-9.

 

Long-QT syndrome is an inherited cardiac channelopathy characterized by delayed repolarization, risk of life-threatening arrhythmia, and significant clinical variability even within families. Three single-nucleotide polymorphisms (SNPs) in the 3' untranslated region of KCNQ1 were ...

Last Updated: 17 Aug 2016

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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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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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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: 28 Sep 2016

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5-HT3 Antagonists (Antiemetics) and Cardiac Safety
 

Status: Recruiting

Condition Summary: Adverse Reaction to Other Drugs and Medicines

 

Last Updated: 2 Nov 2017

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