Cardiac arrest under anaesthesia in a child with previously undiagnosed Jervell and Lange-Nielsen syndrome.

A 7-year-old Sikh boy with a history of syncopal attacks and congenital deafness was admitted for elective adenoidectomy and examination of his ears under general anaesthesia. Immediately after induction of anaesthesia an ECG demonstrated T wave inversion in the CM5 lead. The child subsequently developed multifocal ventricular extrasystoles and later, ventricular fibrillation. Defibrillation was achieved using two 50 J DC shocks. A 12-lead ECG performed later demonstrated a prolonged Q-Tc interval (0.52 s). The child was diagnosed as having the Jervell and Lange-Nielsen syndrome.