CALM3 mutation associated with long QT syndrome

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Novel CALM3 mutations in pediatric long QT syndrome patients support a CALM3-specific calmodulinopathy

Marie-A. Chaix, MD, MSc, Tamara T. Koopmann, PhD, Philippe Goyette, PhD, Azadeh Alikashani, MSc, Frédéric Latour, MSc, Meena Fatah, HBSc, Robert M. Hamilton, MD, John D. Rioux, PhD From the Montreal Heart Institute, Montreal, Quebec, Canada, Université de Montréal, Montreal, Quebec, Canada, and Physiology and Experimental Medicine, The Hospital for Sick Children & Research Institute, Toronto, O...

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KCNE1 and KCNE2 variants in Patients with Long QT Syndrome

Introduction: Long QT syndrome (LQTS) is a type of ventricular arrhythmia characterized by prolonged QT intervals on electrocardiogram or delay in ventricular repolarization and it can lead to syncope, seizure and sudden cardiac death. Here, KCNE1 and KCNE2 variants are studied among Iranian affected families with this syndrome. Materials and Methods: Fifty patients referring to Rajaei Cardiov...

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Long-QT syndrome in a family with a KCNH2 mutation

Long-QT syndrome (LQTS) is an inherited ion channelopathy resulting in abnormal ventricular repolarization and abnormal prolongation of the QT interval on the electrocardiogram. Clinical features vary, from asymptomatic individuals to those with presyncope, life threatening ventricular arrhythmias and sudden cardiac death (SCD). This case report describes a family with a mutation of the KCNH2 g...

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Isoform-Specific Dominant-Negative Effects Associated with hERG1 G628S Mutation in Long QT Syndrome

BACKGROUND Mutations in the human ether-a-go-go-related gene 1 (hERG1) cause type 2 long QT syndrome (LQT2). The hERG1 gene encodes a K(+) channel with properties similar to the rapidly activating delayed rectifying K(+) current in the heart. Several hERG1 isoforms with unique structural and functional properties have been identified. To date, the pathogenic mechanisms of LQT2 mutations have be...

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Novel mutation in the KCNH2 gene associated with long QT syndrome.

A 37-year-old man was admitted to our department after an episode of rapid regular palpitations, triggered by emotional stress. He had no previous symptoms and was not taking any medication. There was no relevant family history. The first two electrocardiograms documented sinus rhythm and a pattern of abnormal repolarization with STsegment elevation. The corrected QT interval (QTc) was between ...

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ژورنال

عنوان ژورنال: Heart Rhythm

سال: 2015

ISSN: 1547-5271

DOI: 10.1016/j.hrthm.2014.10.035