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

Sex is a moderator of the association between NOS1AP sequence variants and QTc in two long QT syndrome founder populations: a pedigree-based measured genotype association analysis.
 

Author(s): Annika Winbo, Eva-Lena Stattin, Ida Maria Westin, Anna Norberg, Johan Persson, Steen M Jensen, Annika Rydberg

Journal:

 

Sequence variants in the NOS1AP gene have repeatedly been reported to influence QTc, albeit with moderate effect sizes. In the long QT syndrome (LQTS), this may contribute to the substantial QTc variance seen among carriers of identical pathogenic sequence variants. Here we assess ...

Last Updated: 19 Jul 2017

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A novel KCNQ1 nonsense variant in the isoform-specific first exon causes both jervell and Lange-Nielsen syndrome 1 and long QT syndrome 1: a case report.
 

Author(s): Motoi Nishimura, Marehiko Ueda, Ryota Ebata, Emi Utsuno, Takuma Ishii, Kazuyuki Matsushita, Osamu Ohara, Naoki Shimojo, Yoshio Kobayashi, Fumio Nomura

Journal:

 

According to previous KCNQ1 (potassium channel, voltage gated, KQT-like subfamily, member 1) gene screening studies, missense variants, but not nonsense or frame-shift variants, cause the majority of long QT syndrome (LQTS; Romano-Ward syndrome [RWS]) 1 cases. Several missense variants ...

Last Updated: 9 Jun 2017

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TRPM4 non-selective cation channel variants in long QT syndrome.
 

Author(s): Thomas Hof, Hui Liu, Laurent Sallé, Jean-Jacques Schott, Corinne Ducreux, Gilles Millat, Philippe Chevalier, Vincent Probst, Romain Guinamard, Patrice Bouvagnet

Journal:

 

Long QT syndrome (LQTS) is an inherited arrhythmic disorder characterized by prolongation of the QT interval, a risk of syncope, and sudden death. There are already a number of causal genes in LQTS, but not all LQTS patients have an identified mutation, which suggests LQTS unknown genes.

Last Updated: 19 Mar 2017

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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 48 free, full-text review articles on human participants. First 3 results:

A Tale of 2 Diseases: The History of Long-QT Syndrome and Brugada Syndrome.
 

Author(s): Ofer Havakuk, Sami Viskin

Journal: J. Am. Coll. Cardiol.. 2016 Jan;67(1):100-8.

 

The Brugada syndrome (BrS) and long-QT syndrome (LQTS) present as congenital or acquired disorders with diagnostic electrocardiograms (ST-segment elevation and prolonged QT interval, respectively) and increased risk for malignant arrhythmias. Our understanding of the 2 disease forms ...

Last Updated: 14 Jan 2016

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The link between abnormal calcium handling and electrical instability in acquired long QT syndrome--Does calcium precipitate arrhythmic storms?
 

Author(s): Jan Němec, Jong J Kim, Guy Salama

Journal: Prog. Biophys. Mol. Biol.. 2016 Jan;120(1-3):210-21.

 

Release of Ca(2+) ions from sarcoplasmic reticulum (SR) into myocyte cytoplasm and their binding to troponin C is the final signal form myocardial contraction. Synchronous contraction of ventricular myocytes is necessary for efficient cardiac pumping function. This requires both shuttling ...

Last Updated: 26 Mar 2016

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A Systematic Review on the Cost-Effectiveness of Genetic and Electrocardiogram Testing for Long QT Syndrome in Infants and Young Adults.
 

Author(s): Fernando Matias Gonzalez, Maria Assunta Veneziano, Anna Puggina, Stefania Boccia

Journal: Value Health. 2015 Jul;18(5):700-8.

 

Recent improvements in the identification of the genetic basis of long QT syndrome (LQTS) have led to significant changes in the diagnosis and management of this life-threatening condition. Genetic and electrocardiogram (ECG) tests are the most relevant examples among testing strategies ...

Last Updated: 22 Aug 2015

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Long QT Syndrome Screening in Newborns
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 26 Sep 2016

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The Canadian National Long QT Syndrome Registry
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 16 May 2016

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Prospective Identification of Long QT Syndrome in Fetal Life
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 22 Aug 2016

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