A Case of Long QT Syndrome Type 3 Aggravated by Beta-Blockers and Alleviated by Mexiletine: The Role of Epinephrine Provocation Test.
10.3349/ymj.2013.54.2.529
- Author:
Junbeom PARK
1
;
Sook Kyoung KIM
;
Hui Nam PAK
Author Information
1. Department of Cardiology, Yonsei University Health System, Seoul, Korea. hnpak@yuhs.ac
- Publication Type:Case Reports ; Research Support, Non-U.S. Gov't
- Keywords:
Long QT syndrome type 3;
torsade de pointes;
beta-blocker;
mexiletine;
SCN5A
- MeSH:
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
- From:Yonsei Medical Journal
2013;54(2):529-533
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
