1.A Case of Long QT Syndrome Type 3 Aggravated by Beta-Blockers and Alleviated by Mexiletine: The Role of Epinephrine Provocation Test.
Junbeom PARK ; Sook Kyoung KIM ; Hui Nam PAK
Yonsei Medical Journal 2013;54(2):529-533
Long QT syndrome (LQTs) is an uncommon genetic disease causing sudden cardiac death with Torsade de Pointes (TdP). The first line drug treatment has been known to be beta-blocker. We encountered a 15-year-old female student with LQTs who had prolonged QTc and multiple episodes of syncope or agonal respiration during sleep. Although her T wave morphology in surface electrocardiography resembled LQTs type 1, her clinical presentation was unusual. During the epinephrine test, TdP was aggravated during beta-blocker medication, but alleviated by sodium channel blocker (mexiletine). Therefore, she underwent implantable cardioverter defibrillator implantation.
Adolescent
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Adrenergic beta-Antagonists/*adverse effects/therapeutic use
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Defibrillators, Implantable
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Diagnosis, Differential
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Diagnostic Techniques, Cardiovascular
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Epinephrine/*diagnostic use
;
Female
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Humans
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Long QT Syndrome/classification/*diagnosis/genetics/therapy
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Mexiletine/*therapeutic use
;
Pedigree
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*Syncope