Abstract
Long QT syndrome is not rare syndrome, but it is necessary to pay attention to ventricular fibrillation in order to avoid adverse outcomes. We herein report the case of a 12-year-old female who developed ventricular fibrillation while swimming in her elementary school's pool. The fibrillation resulted in sudden onset cardiac arrest. Her teacher immediately performed cardiac resuscitation with an automated external defibrillator. She soon had a return of spontaneous circulation, however, she did not recover consciousness, and was transferred to our hospital after intratracheal intubation by doctor. Upon arrival at the hospital, her consciousness level was deep coma, her bilateral pupils were 3 mm in diameter, and her blood pressure was 136/74mmHg, her pulse rate was 84/minute, and her respiratory rate was 19/ minute. An electrocardiogram displayed an extended QT time after correction. She recovered with no neurological deficits after brain hypothermia therapy at 33°C for 24 hours. She was diagnosed to have long QT syndrome, and was therefore suspected to have Romano-Ward syndrome because of her family history. As a result, she received an implantable cardioverter defibrillator on the seventh hospital day. She was uneventfully discharged on the 14 hospital day. The presence of long QT syndrome in a young subject is generally suspected to be due to a congenital disease such as Romano-Ward syndrome. Romano-Ward syndrome is an autosomal dominant disease, therefore it is necessary to perform genetic diagnosis to confirm the presence of the defective gene. Implantable cardioverter defibrillator insertion is indispensable in the high risk patients for ventricular fibrillation.