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KCNH2 변이가 확인된 2형 선천성 QT 간격 연장 증후군 1예

Type 2 Congenital Long QT Syndrome with KCNH2 Mutation

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The Medical Journal of Chosun University (조선대학교 의대논문집)
조선대학교 의학연구원 (Institute of Medical Science, Chosun University)
초록

A 41-year-old woman arrived at our emergency department with semicomatous mentality due to aborted cardiac arrest from ventricular fibrillation, which occurred during resting state. Prolonged QT interval in condition of hypokalemia and hypomagnesemia was observed on initial electrocardiography. However, even after normalization of serum potassium, magnesium and mentality, QT prolongation was sustained. In addition, genetic analysis showed KCNH2 mutation which was consistent with type 2 congenital long QT syndrome (LQTS). In this case, the patient was treated with an implantable cardioverter defibrillator and genotype specific medical therapy with potassium channel opener.

저자
  • 김병기(성균관대학교 의과대학 삼성창원병원) | Byeong-Ki Kim
  • 김기훈(성균관대학교 의과대학 삼성창원병원) | Ki-Hoon Kim
  • 박만제(성균관대학교 의과대학 삼성창원병원) | Man-Je Park
  • 옥혜성(성균관대학교 의과대학 삼성창원병원) | Hea-Sung Ock
  • 이현수(성균관대학교 의과대학 삼성창원병원) | Hyoun-Soo Lee
  • 오주현(성균관대학교 의과대학 삼성창원병원) | Ju-Hyun Oh
  • 강구현(성균관대학교 의과대학 삼성창원병원) | Gu-Hyun Kang Corresponding author