Long QT syndrome 1

Common Name(s)

Long QT syndrome 1

Romano-Ward syndrome is the most common form of inherited long QT syndrome. Symptoms include arrhythmia, fainting, cardiac arrest, and sudden death. There are six different types of this syndrome, long QT 1 through 6. Each type is caused by a change in a different gene. The most prevalent form of long QT syndrome is long QT type 1. Long QT type 1 is caused by changes in the KCNQ1 gene. Romano-Ward syndrome is inherited in an autosomal dominant fashion.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Long QT syndrome 1" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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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 1" returned 37 free, full-text research articles on human participants. First 3 results:

Characterization of a novel KCNQ1 mutation for type 1 long QT syndrome and assessment of the therapeutic potential of a novel IKs activator using patient-specific induced pluripotent stem cell-derived cardiomyocytes.
 

Author(s): Dongrui Ma, Heming Wei, Jun Lu, Dou Huang, Zhenfeng Liu, Li Jun Loh, Omedul Islam, Reginald Liew, Winston Shim, Stuart A Cook

Journal:

 

Type 1 long QT syndrome (LQT1) is a common type of cardiac channelopathy associated with loss-of-function mutations of KCNQ1. Currently there is a lack of drugs that target the defected slowly activating delayed rectifier potassium channel (IKs). With LQT1 patient-specific human induced ...

Last Updated: 19 Apr 2015

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Autonomic control of heart rate and QT interval variability influences arrhythmic risk in long QT syndrome type 1.
 

Author(s): Alberto Porta, Giulia Girardengo, Vlasta Bari, Alfred L George, Paul A Brink, Althea Goosen, Lia Crotti, Peter J Schwartz

Journal: J. Am. Coll. Cardiol.. 2015 Feb;65(4):367-74.

 

A puzzling feature of the long QT syndrome (LQTS) is that family members carrying the same mutation often have divergent symptoms and clinical outcomes.

Last Updated: 30 Jan 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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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 1" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Comparison Between Epinephrine and Exercise Test in QT Long Syndrome Patients
 

Status: Recruiting

Condition Summary: Long QT Syndrome Type 1 or 2

 

Last Updated: 23 May 2014

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Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy
 

Status: Not yet recruiting

Condition Summary: Metabolism, Inborn Errors; Lipid Metabolism, Inborn Errors; Carbohydrate Metabolism, Inborn Errors; Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency; Glycogenin-1 Deficiency (Glycogen Storage Disease Type XV); Carnitine Palmitoyl Transferase 2 Deficiency; VLCAD Deficiency; Medium-chain Acyl-CoA Dehydrogenase Deficiency; Multiple Acyl-CoA Dehydrogenase Deficiency; Carnitine Transporter Deficiency; Neutral Lipid Storage Disease; Glycogen Storage Disease Type II; Glycogen Storage Disease Type III; Glycogen Storage Disease Type IV; Glycogen Storage Disease Type V; Muscle Phosphofructokinase Deficiency; Phosphoglucomutase 1 Deficiency; Phosphoglycerate Mutase Deficiency; Phosphoglycerate Kinase Deficiency; Phosphorylase Kinase Deficiency; Beta Enolase Deficiency; Lactate Dehydrogenase Deficiency; Glycogen Synthase Deficiency

 

Last Updated: 15 Dec 2015

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Pharmacokinetics of Eleclazine in Adults With Normal and Impaired Hepatic Function
 

Status: Recruiting

Condition Summary: Long QT Syndrome

 

Last Updated: 5 Feb 2016

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