Jervell and Lange–Nielsen Syndrome Treated with Implantable Defibrillator—A Case Report

Jervell and Lange–Nielsen syndrome is an extremely rare disease. We report a case of inherited long QT syndrome in a 13-year-old boy with bilateral deafness and recurrent syncope. Because his older sister had died at school suddenly when she was 14 years old, the patient’s family was encouraged to undergo electrocardiographic testing to check for QT prolongation. No signs of heart disease could be detected by physical examination, X-ray examination, or echo Doppler study. He had relative bradycardia before the initiation of a beta blocker. A dual-chamber, automated, internal cardioverter defibrillator was implanted for protection from sudden death and to support the heart rate with a large dose of beta locker.