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 ; Adrenergic beta-Antagonists/*adverse effects/therapeutic use ; Defibrillators, Implantable ; Diagnosis, Differential ; Diagnostic Techniques, Cardiovascular ; Epinephrine/*diagnostic use ; Female ; Humans ; Long QT Syndrome/classification/*diagnosis/genetics/therapy ; Mexiletine/*therapeutic use ; Pedigree ; *Syncope