Genotype- and Mutation Site–Specific QT Adaptation During Exercise, Recovery, and Postural Changes in Children With Long-QT Syndrome

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Genotype- and mutation site-specific QT adaptation during exercise, recovery, and postural changes in children with long-QT syndrome.

BACKGROUND Exercise stress testing has shown diagnostic utility in adult patients with long-QT syndrome (LQTS); however, the QT interval adaptation in response to exercise in pediatric patients with LQTS has received little attention. METHODS AND RESULTS One-hundred fifty-eight patients were divided into 3 groups: Those with LQTS type 1 (LQT1) or LQTS type 2 (LQT2) and normal control subjects...

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Rate adaptation of QT intervals during and after exercise in children with congenital long QT syndrome.

OBJECTIVES To improve the diagnostic criteria of the congenital long QT syndrome in borderline cases we examined rate adaptation of ventricular repolarization phases during exercise and subsequent recovery in children with the long QT syndrome and controls. METHODS Nineteen children with definite long QT syndrome and 19 healthy controls underwent exercise testing. QT intervals were measured t...

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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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Genotype and severity of long QT syndrome.

OBJECTIVES To describe the state of the art of our understanding of the long QT syndromes and to provide the genetic correlation of clinical severity of patients with this disorder. DATE SOURCES In this review, we outline data that were obtained from work in our laboratory, as well as information reported in the literature. STUDY SELECTION The information in this review spans the last decad...

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Long QT Syndrome in Children

he advent of genetic testing and the implantable ardioverter-defibrillator (ICD) have so revolutionized the utlook for patients with long QT that the cardiac commuity needs to revise its thinking about long QT syndrome LQTS). This is the underlying message of a report by theridge et al. (1) in this issue of the Journal. Etheridge et al. (1) studied 128 children with LQTS. bout one-half of their...

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ژورنال

عنوان ژورنال: Circulation: Arrhythmia and Electrophysiology

سال: 2011

ISSN: 1941-3149,1941-3084

DOI: 10.1161/circep.111.963330