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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Condition Specific Organizations

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

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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 38 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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Enhanced effects of isoflurane on the long QT syndrome 1-associated A341V mutant.
 

Author(s): Ikuomi Mikuni, Carlos G Torres, Tania Bakshi, Akihito Tampo, Brian E Carlson, Martin W Bienengraeber, Wai-Meng Kwok

Journal: Anesthesiology. 2015 Apr;122(4):806-20.

 

The impact of volatile anesthetics on patients with inherited long QT syndrome (LQTS) is not well understood. This is further complicated by the different genotypes underlying LQTS. No studies have reported on the direct effects of volatile anesthetics on specific LQTS-associated ...

Last Updated: 18 Mar 2015

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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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Symptoms, Diagnosis, and Treatment

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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: 12 Apr 2016

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