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 26 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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Human iPS cell model of type 3 long QT syndrome recapitulates drug-based phenotype correction.
 

Author(s): Daniela Malan, Miao Zhang, Birgit Stallmeyer, Jovanca Müller, Bernd K Fleischmann, Eric Schulze-Bahr, Philipp Sasse, Boris Greber

Journal: Basic Res. Cardiol.. 2016 Mar;111(2):14.

 

Long QT syndrome is a potentially life-threatening disease characterized by delayed repolarization of cardiomyocytes, QT interval prolongation in the electrocardiogram, and a high risk for sudden cardiac death caused by ventricular arrhythmia. The genetic type 3 of this syndrome (LQT3) ...

Last Updated: 25 Jan 2016

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Pronounced Shortening of QT Interval With Mexiletine Infusion Test in Patients With Type 3 Congenital Long QT Syndrome.
 

Author(s): Moritoshi Funasako, Takeshi Aiba, Kohei Ishibashi, Ikutaro Nakajima, Koji Miyamoto, Yuko Inoue, Hideo Okamura, Takashi Noda, Shiro Kamakura, Toshihisa Anzai, Teruo Noguchi, Satoshi Yasuda, Yoshihiro Miyamoto, Kengo Fukushima Kusano, Hisao Ogawa, Wataru Shimizu

Journal: Circ. J.. 2016 ;80(2):340-5.

 

Mexiletine is often used for medical therapy in LQT3 patients, however, the usefulness of mexiletine infusion test for LQT3 patients has not been reported. The aim of this study was to evaluate the usefulness of mexiletine infusion test for detecting LQT3 patients.

Last Updated: 25 Jan 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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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: 28 Sep 2016

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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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