Long QT syndrome 2

Common Name(s)

Long QT syndrome 2

Congenital long QT syndrome is electrocardiographically characterized by a prolonged QT interval and polymorphic ventricular arrhythmias (torsade de pointes). These cardiac arrhythmias may result in recurrent syncope, seizure, or sudden death ({7:Jongbloed et al., 1999}). For a discussion of genetic heterogeneity of long QT syndrome, see LQT1 ({192500}).
 

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Condition Specific Organizations

Following organizations serve the condition "Long QT syndrome 2" 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 2" returned 37 free, full-text research articles on human participants. First 3 results:

Generation of human induced pluripotent stem cell line from a patient with a long QT syndrome type 2.
 

Author(s): Azra Fatima, Dina Ivanyuk, Stefan Herms, Stefanie Heilmann-Heimbach, Orla O'Shea, Charlotte Chapman, Zsuszanna Izsvák, Martin Farr, Jürgen Hescheler, Tomo Šarić

Journal: Stem Cell Res. 2016 Mar;16(2):304-7.

 

We report here the generation of human iPS cell line UKKi009-A from dermal fibroblasts of a patient carrying heterozygous mutation c.3035-3045delTCCCTCGATGC, p.Leu1012Pro (fs*55) in KCNH2 gene leading to long QT syndrome type 2 (LQT2). We used the Sleeping Beauty transposon-based ...

Last Updated: 27 Jun 2016

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Structure of the Cyclic Nucleotide-Binding Homology Domain of the hERG Channel and Its Insight into Type 2 Long QT Syndrome.
 

Author(s): Yan Li, Hui Qi Ng, Qingxin Li, CongBao Kang

Journal:

 

The human ether-à-go-go related gene (hERG) channel is crucial for the cardiac action potential by contributing to the fast delayed-rectifier potassium current. Mutations in the hERG channel result in type 2 long QT syndrome (LQT2). The hERG channel contains a cyclic nucleotide-binding ...

Last Updated: 30 Mar 2016

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Association of the hERG mutation with long-QT syndrome type 2, syncope and epilepsy.
 

Author(s): Guoliang Li, Rui Shi, Jine Wu, Wenqi Han, Aifeng Zhang, Gong Cheng, Xiaolin Xue, Chaofeng Sun

Journal: Mol Med Rep. 2016 Mar;13(3):2467-75.

 

Mutations in the human ether‑à‑go‑go‑related gene (hERG) are responsible for long‑QT syndrome (LQTS) type 2 (LQT2). In the present study, a heterozygous missense mutation (A561V) linked to LQT2, syncope and epilepsy was identified in the S5/pore region of the hERG protein. ...

Last Updated: 7 Apr 2016

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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 2" returned 1 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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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: 4 Oct 2016

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