Abstract
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.
