Epinephrine QT Stress Testing in the Evaluation of Congenital Long-QT Syndrome

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Epinephrine QT stress testing in the evaluation of congenital long-QT syndrome: diagnostic accuracy of the paradoxical QT response.

BACKGROUND A paradoxical increase in the uncorrected QT interval during infusion of low-dose epinephrine appears pathognomonic for type 1 long-QT syndrome (LQT1). We sought to determine the diagnostic accuracy of this response among patients referred for clinical evaluation of congenital long-QT syndrome (LQTS). METHODS AND RESULTS From 1999 to 2002, 147 genotyped patients (125 untreated and ...

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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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Prevalence of the congenital long-QT syndrome.

BACKGROUND The prevalence of genetic arrhythmogenic diseases is unknown. For the long-QT syndrome (LQTS), figures ranging from 1:20 000 to 1:5000 were published, but none was based on actual data. Our objective was to define the prevalence of LQTS. METHODS AND RESULTS In 18 maternity hospitals, an ECG was performed in 44 596 infants 15 to 25 days old (43 080 whites). In infants with a correct...

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Medical Policy Subject: Genetic Testing for Congenital Long Qt Syndrome

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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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ژورنال

عنوان ژورنال: Circulation

سال: 2006

ISSN: 0009-7322,1524-4539

DOI: 10.1161/circulationaha.105.600445