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: 4 May 2015

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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: 23 Apr 2015

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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: 4 May 2015

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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: 23 Apr 2015

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

Assessment of the predictive accuracy of five in silico prediction tools, alone or in combination, and two metaservers to classify long QT syndrome gene mutations.
 

Author(s): Ivone U S Leong, Alexander Stuckey, Daniel Lai, Jonathan R Skinner, Donald R Love

Journal:

 

Long QT syndrome (LQTS) is an autosomal dominant condition predisposing to sudden death from malignant arrhythmia. Genetic testing identifies many missense single nucleotide variants of uncertain pathogenicity. Establishing genetic pathogenicity is an essential prerequisite to family ...

Last Updated: 20 May 2015

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Large-scale mutational analysis of Kv11.1 reveals molecular insights into type 2 long QT syndrome.
 

Author(s): Corey L Anderson, Catherine E Kuzmicki, Ryan R Childs, Caleb J Hintz, Brian P Delisle, Craig T January

Journal:

 

It has been suggested that deficient protein trafficking to the cell membrane is the dominant mechanism associated with type 2 Long QT syndrome (LQT2) caused by Kv11.1 potassium channel missense mutations, and that for many mutations the trafficking defect can be corrected pharmacologically. ...

Last Updated: 24 Nov 2014

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Long QT syndrome associated with adrenal insufficiency in a patient with isolated adrenocorticotropic hormone deficiency.
 

Author(s): Kenta Kanamori, Risa Yamashita, Kenta Tsutsui, Masumi Hara, Yuji Murakawa

Journal: Intern. Med.. 2014 ;53(20):2329-31.

 

QT prolongation and Torsades de Pointes were observed in a 44-year-old woman who had adrenal insufficiency caused by isolated adrenocorticotropic hormone deficiency. Although she had several risk factors for QT prolongation, we concluded that the adrenal insufficiency contributed ...

Last Updated: 16 Oct 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 50 free, full-text review articles on human participants. First 3 results:

Genetic and clinical advances in congenital long QT syndrome.
 

Author(s): Yuka Mizusawa, Minoru Horie, Arthur A M Wilde

Journal: Circ. J.. 2014 ;78(12):2827-33.

 

Congenital long QT syndrome (LQTS) is an inherited arrhythmia syndrome characterized by a prolonged QT interval on the 12-lead ECG, torsades de pointes and a higher chance of sudden cardiac death. LQTS segregates in a Mendelian fashion, which includes Romano-Ward syndrome with an ...

Last Updated: 25 Nov 2014

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Genetics of long QT syndrome.
 

Author(s): David J Tester, Michael J Ackerman

Journal: Methodist Debakey Cardiovasc J. ;10(1):29-33.

 

Long QT syndrome (LQTS) is a potentially life-threatening cardiac arrhythmia characterized by delayed myocardial repolarization that produces QT prolongation and increased risk for torsades des pointes (TdP)-triggered syncope, seizures, and sudden cardiac death (SCD) in an otherwise ...

Last Updated: 16 Jun 2014

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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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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

The Canadian National Long QT Syndrome Registry
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 20 Apr 2015

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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: 25 Mar 2015

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