Contribution of a KCNH2 variant in genotyped long QT syndrome: Romano–Ward syndrome under double mutations and acquired long QT syndrome under heterozygote

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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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The QT interval is the electrocardiographic manifestation of ventricular repolarization, is variable under physiologic conditions, and is measurably prolonged by many drugs. Rarely, however, individuals with normal base-line intervals may display exaggerated QT interval prolongation, and the potentially fatal polymorphic ventricular tachycardia torsade de pointes, with drugs or other environmen...

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Introduction The Long QT syndrome (LQTS) can be inherited or acquired and is of particular interest and concern at present. Patients with LQTS are predisposed to the ventricular tachyarrhythmia torsade de pointes (TdP) which causes syncope and sudden death. Inherited LQTS is the prototype of the "primary cardiac arrhythmias" or "cardiac ion channelopathies". The study of inherited LQTS has prov...

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ژورنال

عنوان ژورنال: Journal of Cardiology

سال: 2017

ISSN: 0914-5087

DOI: 10.1016/j.jjcc.2016.09.010