Aborted Sudden Cardiac Death Associated with Short QT Syndrome

A 43-year-old male was transferred to our institute. His heart rhythm on admission was ventricular fibrillation (VF) which was successfully defibrillated with a direct current shock (DC). A diagnosis of short QT syndrome (SQTS) was made on the basis of an abnormally short QT interval of 280 ms during the sinus rhythm. During treatment for mild total hypothermia, VF recurred repeatedly necessitating DCs. Nifekalant at a dose of 0.3 mg/kg was intravenously administered, the QT interval was prolonged from 280 to 370 ms and VF no longer recurred. Subsequently the patient underwent implantation of an implantable cardioverter defibrillator.