Congenital long QT syndrome (LQTS) is frequently misdiagnosed and maltreated due to its similar presentations to
many disorders, most notably idiopathic epilepsy. The syndrome is characterized by a prolonged QT interval and
T-wave morphology abnormalities in association with torsade de pointes. The heart is structurally and functionally
normal.
We report a 17-year-old male teenager who was initially treated as idiopathic epilepsy for 5 years. Clinical
presentations, series examinations and inadequate response to anti-convulsant treatment lead to the final diagnosis
of congenital long QT syndrome. Beta-blocker treatment was the treatment of choice, but it was complicated by
severe bradycardia. Ademand-type atrial chamber pacemaker (AAIR) was placed initially, but it was not sufficient.
Dual-chamber implantable cardioverter-defibrillator (ICD) was the best solution for this patient.
