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}).
 

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

The role of CAV3 in long-QT syndrome: clinical and functional assessment of a caveolin-3/Kv11.1 double heterozygote versus caveolin-3 single heterozygote.
 

Author(s): Paula L Hedley, Jørgen K Kanters, Maja Dembic, Thomas Jespersen, Lasse Skibsbye, Frederik H Aidt, Ole Eschen, Claus Graff, Elijah R Behr, Sarah Schlamowitz, Valerie Corfield, William J McKenna, Michael Christiansen

Journal: Circ Cardiovasc Genet. 2013 Oct;6(5):452-61.

 

Mutations in CAV3, coding for caveolin-3, the major constituent scaffolding protein of cardiac caveolae, have been associated with skeletal muscle disease, cardiomyopathy, and most recently long-QT syndrome (LQTS) and sudden infant death syndrome. We examined the occurrence of CAV3 ...

Last Updated: 16 Oct 2013

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The interaction of caveolin 3 protein with the potassium inward rectifier channel Kir2.1: physiology and pathology related to long qt syndrome 9 (LQT9).
 

Author(s): Ravi Vaidyanathan, Amanda L Vega, Chunhua Song, Qing Zhou, Bi-Hua Tan, Bihua Tan, Stuart Berger, Jonathan C Makielski, Lee L Eckhardt

Journal: J. Biol. Chem.. 2013 Jun;288(24):17472-80.

 

Mutations in CAV3 cause LQT syndrome 9 (LQT9). A previously reported LQT9 patient had prominent U waves on ECG, a feature that has been correlated with Kir2.1 loss of function. Our objective was to determine whether caveolin 3 (Cav3) associates with Kir2.1 and whether LQT9-associated ...

Last Updated: 17 Jun 2013

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Ventricular dysfunction in a family with long QT syndrome type 3.
 

Author(s): Yoran M Hummel, Arthur A M Wilde, Adriaan A Voors, Silvia Bugatti, Hans L Hillege, Maarten P van den Berg

Journal: Europace. 2013 Oct;15(10):1516-21.

 

Long QT syndrome (LQTS) type 3 is characterized by prolonged ventricular repolarization due to persistent sodium inward current secondary to a mutation in SCN5a, the gene encoding for the α-subunit of the sodium channel. We speculated that by disrupting calcium homeostasis the persistent ...

Last Updated: 27 Sep 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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Clinical Trial Information This information is provided by ClinicalTrials.gov

Worm Study: Modifier Genes in Sudden Cardiac Death
 

Status: Not yet recruiting

Condition Summary: Brugada Syndrome; Long QT Syndrome 3

 

Last Updated: 26 May 2014

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Efficacy Study of Sodium Channel Blocker in LQT3 Patients
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 11 Dec 2013

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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: 20 May 2014

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