KMID : 1148020130380020081
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Á¶¼±´ëÇб³ÀÇ´ë³í¹®Áý 2013 Volume.38 No. 2 p.81 ~ p.84
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Type 2 Congenital Long QT Syndrome with KCNH2 Mutation
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Kim Byeong-Ki
Kim Ki-Hoon Park Man-Je Ok Hea-Sung Lee Hyoun-Soo Oh Joo-Hyun Kang Gu-Hyun
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Abstract
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A 41-year-old woman arrived at our emergency department with semicomatous mentality due to aborted cardiac arrest fromventricular fibrillation, which occurred during resting state. Prolonged QT interval in condition of hypokalemia and hypomagnesemiawas observed on initial electrocardiography. However, even after normalization of serum potassium, magnesiumand mentality, QT prolongation was sustained. In addition, genetic analysis showed KCNH2 mutation which was consistentwith type 2 congenital long QT syndrome (LQTS). In this case, the patient was treated with an implantable cardioverterdefibrillator and genotype specific medical therapy with potassium channel opener.
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KEYWORD
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Long QT syndrome, Torsades de pointes, Defibrillators, Implantable sudden cardiac death or VF
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