Diagnostic Miscues in 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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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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Efficient genotyping for congenital long QT syndrome.

WITHIN THE PAST DECADE, INVESTIGATORS HAVE uncovered the genetic basis for multiple long QT syndromes (LQTSs). These include LQT1, in which mutations in the gene KCNQ1 lead to abnormal IKs, a slow delayed-rectifier potassium current; LQT2, in which mutations in the gene KCNH2 lead to abnormal IKr, a rapid delayed-rectifier potassium current; and LQT3, in which mutations in the gene SCN5A lead t...

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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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ژورنال

عنوان ژورنال: Circulation

سال: 2007

ISSN: 0009-7322,1524-4539

DOI: 10.1161/circulationaha.106.661082