QT Adaptation and Intrinsic QT Variability in Congenital Long QT Syndrome

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QT Adaptation and Intrinsic QT Variability in Congenital Long QT Syndrome

BACKGROUND Increased variability of QT interval (QTV) has been linked to arrhythmias in animal experiments and multiple clinical situations. Congenital long QT syndrome (LQTS), a pure repolarization disease, may provide important information on the relationship between delayed repolarization and QTV. METHODS AND RESULTS Twenty-four-hour Holter monitor tracings from 78 genotyped congenital LQT...

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Congenital long QT syndrome

Congenital long QT syndrome (LQTS) is a hereditary cardiac disease characterized by a prolongation of the QT interval at basal ECG and by a high risk of life-threatening arrhythmias. Disease prevalence is estimated at close to 1 in 2,500 live births. The two cardinal manifestations of LQTS are syncopal episodes, that may lead to cardiac arrest and sudden cardiac death, and electrocardiographic ...

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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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Diagnostic miscues in congenital long-QT syndrome.

BACKGROUND Long-QT syndrome (LQTS) is a potentially lethal cardiac channelopathy that can be mistaken for palpitations, neurocardiogenic syncope, and epilepsy. Because of increased physician and public awareness of warning signs suggestive of LQTS, there is the potential for LQTS to be overdiagnosed. We sought to determine the agreement between the dismissal diagnosis from an LQTS subspecialty ...

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Congenital Short QT Syndrome

Long QT intervals in the ECG have long been associated with sudden cardiac death. The congenital long QT syndrome was first described in individuals with structurally normal hearts in 1957.1 Little was known about the significance of a short QT interval. In 1993, after analyzing 6693 consecutive Holter recordings Algra et al concluded that an increased risk of sudden death was present not only ...

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ژورنال

عنوان ژورنال: Journal of the American Heart Association

سال: 2015

ISSN: 2047-9980

DOI: 10.1161/jaha.115.002395