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Highlights the critical importance of recognizing congenital deafness and syncope as warning signs for Jervell and Lange-Nielsen syndrome to prevent life-threatening perioperative arrhythmias.
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.
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J.J. Holland (1993) studied this question.
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