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KMID : 1011920130140040006
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International Journal of Arrhythmia
2013 Volume.14 No. 4 p.6 ~ p.13
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Congenital Long QT Syndrome
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Huh June
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Abstract
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Long QT syndrome (LQTS) is a well-known inheritable channelopathy that causes fatal ventricular arrhythmias. With the advancement of genetic science, the treatment of LQTS has become a paradigm for the use of genetic information inclinical practice. LQTS can present with incomplete penetrance and variable expressivity. Ten percent of silent carriers of a disease-causing mutation usually experienced symptoms when they were treated with QT-prolonging drugs. Patients with LQTS and multiple mutations have a greater risk for life-threatening cardiac events than do patients with a single mutation. LQTS tends to be easily overdiagnosed because of overlapping of a corrected QT interval (QTc), the range between normal valuesand LQTS, and lack of complete diagnostic criteria. The first line of treatment for all patients is β-blocker therapy. Left cardiothoracic sympathetic denervation (LCSD) may be a therapeutic adjunct for young patients with LQTS who are not fully protected by β-blocker therapy. In patients with recurrent symptoms during therapy or in those at high risk for arrhythmias, an implantable cardioverter-defibrillator (ICD) should be considered. The most important aspect of the treatment of LQTS is to determine whether the patient is at high or low risk for life-threatening arrhythmias, so that treatment can be tailored accordingly on an individual basis, more or less aggressively
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KEYWORD
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adrenergic beta-antagonists, defibrillators, genetic diseases, implantable, inborn, left cardiothoracic sympathetic denervation, long QT syndrome
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