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

View Details
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

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

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

View Details
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

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

General Resources

Drugs to Avoid List

Listing of drugs to avoid if you have Long QT Syndrome

Updated 29 Apr 2014

Go To URL
SADS Foundation Website

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

Updated 29 Apr 2014

Go To URL
 
 
Top

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

α1-Syntrophin Variant Identified in Drug-Induced Long QT Syndrome Increases Late Sodium Current.
 

Author(s): Jong-Il Choi, Chaojian Wang, Matthew J Thomas, Geoffrey S Pitt

Journal:

 

Drug-induced long-QT syndrome (diLQTS) is often due to drug block of IKr, especially in genetically susceptible patients with subclinical mutations in the IKr-encoding KCHN2. Few variants in the cardiac NaV1.5 Na+ channel complex have been associated with diLQTS. We tested whether ...

Last Updated: 31 Mar 2016

Go To URL
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

Go To URL
In silico screening of the impact of hERG channel kinetic abnormalities on channel block and susceptibility to acquired long QT syndrome.
 

Author(s): Lucia Romero, Beatriz Trenor, Pei-Chi Yang, Javier Saiz, Colleen E Clancy

Journal: J. Mol. Cell. Cardiol.. 2015 Oct;87():271-82.

 

Accurate diagnosis of predisposition to long QT syndrome is crucial for reducing the risk of cardiac arrhythmias. In recent years, drug-induced provocative tests have proved useful to unmask some latent mutations linked to cardiac arrhythmias. In this study we expanded this concept ...

Last Updated: 8 Feb 2016

Go To URL

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

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

Go To URL
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

Go To URL
Sex differences in the mechanisms underlying long QT syndrome.
 

Author(s): Guy Salama, Glenna C L Bett

Journal: Am. J. Physiol. Heart Circ. Physiol.. 2014 Sep;307(5):H640-8.

 

Sexual dimorphism is a well-established phenomenon, but its degree varies tremendously among species. Since the early days of Einthoven's development of the three-lead galvanometer ECG, we have known there are marked differences in QT intervals of men and women. It required over a ...

Last Updated: 2 Sep 2014

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

 
 
Top

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

Go to URL
The Canadian National Long QT Syndrome Registry
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 16 May 2016

Go to URL
Prospective Identification of Long QT Syndrome in Fetal Life
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 22 Aug 2016

Go to URL