Electrophysiologic Substrate in Congenital Long QT Syndrome
نویسندگان
چکیده
منابع مشابه
Electrophysiologic substrate in congenital Long QT syndrome: noninvasive mapping with electrocardiographic imaging (ECGI).
BACKGROUND Congenital Long QT syndrome (LQTS) is an arrhythmogenic disorder that causes syncope and sudden death. Although its genetic basis has become well-understood, the mechanisms whereby mutations translate to arrhythmia susceptibility in the in situ human heart have not been fully defined. We used noninvasive ECG imaging to map the cardiac electrophysiological substrate and examine whethe...
متن کاملCongenital 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 ...
متن کاملDiagnostic 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 ...
متن کاملElectrophysiologic testing in patients with the long QT syndrome.
Electrophysiologic studies were performed in 15 patients with syncope and/or cardiac arrest who had the long QT syndrome and 11 control subjects who had normal QT intervals. The syndrome was familial in five patients and idiopathic in 10. All patients had a prolonged QT (546 +/- 68 msec, mean +/- SD) and corrected QT (550 +/- 51 msec). Incremental atrial pacing at cycle lengths of 600 to 400 ms...
متن کاملEfficient 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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ژورنال
عنوان ژورنال: Circulation
سال: 2014
ISSN: 0009-7322,1524-4539
DOI: 10.1161/circulationaha.114.011359