Long QT Syndrome

Common Name(s)

Long QT Syndrome

Long QT syndrome is a disorder of the heart’s electrical activity that can cause sudden, uncontrollable, and irregular heartbeats (arrhythmia), which may lead to sudden death. Long QT syndrome can be detected by electrocardiogram (EKG). It can be caused by a variety of different gene mutations (changes). It can also be acquired (noninherited) and may be brought on by certain medicines and other medical conditions.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

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

Kids with Heart National Association for Children's Heart Disorders

Our organization was formed in 1985 with the main mission of providing support, information, resources, and education for the families of the children living with congenital heart defects and to promote public awareness of the issues that these families live with on a day to day basis.

Last Updated: 30 Oct 2012

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SADS (Sudden Arrhythmia Death Syndromes) Foundation

Our mission is to save the lives of young people who are genetically predisposed to sudden death due to cardiac arrhythmia and to provide education and support to families and the medical community.

Last Updated: 29 Apr 2014

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General Support Organizations

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

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

Kids with Heart National Association for Children's Heart Disorders

Our organization was formed in 1985 with the main mission of providing support, information, resources, and education for the families of the children living with congenital heart defects and to promote public awareness of the issues that these families live with on a day to day basis.

http://www.kidswithheart.org

Last Updated: 30 Oct 2012

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SADS (Sudden Arrhythmia Death Syndromes) Foundation

Our mission is to save the lives of young people who are genetically predisposed to sudden death due to cardiac arrhythmia and to provide education and support to families and the medical community.

http://www.StopSADS.org

Last Updated: 29 Apr 2014

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General Support Organizations

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General Resources

Drugs to Avoid List

Listing of drugs to avoid if you have Long QT Syndrome

Updated 29 Apr 2014

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SADS Foundation Website

Information on living with SADS, diagnosis, treatment, prevention, and advocacy/education.

Updated 29 Apr 2014

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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" returned 521 free, full-text research articles on human participants. First 3 results:

Mutations in Danish patients with long QT syndrome and the identification of a large founder family with p.F29L in KCNH2.
 

Author(s): Michael Christiansen, Paula L Hedley, Juliane Theilade, Birgitte Stoevring, Trond P Leren, Ole Eschen, Karina M Sørensen, Anne Tybjærg-Hansen, Lilian B Ousager, Lisbeth N Pedersen, Ruth Frikke-Schmidt, Frederik H Aidt, Michael G Hansen, Jim Hansen, Poul E Bloch Thomsen, Egon Toft, Finn L Henriksen, Henning Bundgaard, Henrik K Jensen, Jørgen K Kanters

Journal:

 

Long QT syndrome (LQTS) is a cardiac ion channelopathy which presents clinically with palpitations, syncope or sudden death. More than 700 LQTS-causing mutations have been identified in 13 genes, all of which encode proteins involved in the execution of the cardiac action potential. ...

Last Updated: 14 Mar 2014

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Long QT molecular autopsy in sudden infant death syndrome.
 

Author(s): Joanna Moira Glengarry, Jackie Crawford, Paul Lowell Morrow, Simon Robert Stables, Donald Roy Love, Jonathan Robert Skinner

Journal: Arch. Dis. Child.. 2014 Jul;99(7):635-40.

 

To describe experience of long QT (LQT) molecular autopsy in sudden infant death syndrome (SIDS).

Last Updated: 3 Sep 2014

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Exome sequencing implicates an increased burden of rare potassium channel variants in the risk of drug-induced long QT interval syndrome.
 

Author(s): Peter Weeke, Jonathan D Mosley, David Hanna, Jessica T Delaney, Christian Shaffer, Quinn S Wells, Sara Van Driest, Jason H Karnes, Christie Ingram, Yan Guo, Yu Shyr, Kris Norris, Prince J Kannankeril, Andrea H Ramirez, Joshua D Smith, Elaine R Mardis, Deborah Nickerson, Alfred L George, Dan M Roden

Journal: J. Am. Coll. Cardiol.. 2014 Apr;63(14):1430-7.

 

The aim of this study was to test the hypothesis that rare variants are associated with drug-induced long QT interval syndrome (diLQTS) and torsades de pointes.

Last Updated: 11 Apr 2014

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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" returned 49 free, full-text review articles on human participants. First 3 results:

Genotype- and phenotype-guided management of congenital long QT syndrome.
 

Author(s): John R Giudicessi, Michael J Ackerman

Journal: Curr Probl Cardiol. 2013 Oct;38(10):417-55.

 

Congenital long QT syndrome (LQTS) is a genetically heterogeneous group of heritable disorders of myocardial repolarization linked by the shared clinical phenotype of QT prolongation on electrocardiogram and an increased risk of potentially life-threatening cardiac arrhythmias. At ...

Last Updated: 7 Oct 2013

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Practical issues in the management of the long QT syndrome: focus on diagnosis and therapy.
 

Author(s): Peter J Schwartz

Journal:

 

The long QT syndrome (LQTS) is a leading cause of sudden death in the young. It is not as rare as previously assumed, given its established prevalence of 1:2,000 live births. It is characterised by prolongation of the QT interval and by the occurrence of syncope, due to torsades-des-pointes ...

Last Updated: 3 Oct 2013

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The long QT syndrome: a transatlantic clinical approach to diagnosis and therapy.
 

Author(s): Peter J Schwartz, Michael J Ackerman

Journal: Eur. Heart J.. 2013 Oct;34(40):3109-16.

 

The mind-boggling progress in the understanding of the molecular mechanisms underlying the long QT syndrome (LQTS) has been the subject of many articles and reviews. Still, when it comes to the management of the patients affected by this life-threatening disorder, too many errors ...

Last Updated: 22 Oct 2013

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Multicenter Evaluation of Children and Young Adults With Genotype Positive Long QT Syndrome
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 29 Oct 2014

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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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Long QT and Hearing Loss Registry
 

Status: Recruiting

Condition Summary: Sensorineural Hearing Loss; Long QT Syndrome

 

Last Updated: 29 Oct 2014

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