Idiopathic long QT syndrome with late onset of bradycardia-dependent and short-coupled variant of torsade de pointes

We report an idiopathic variant of the long QT syndrome in a 72 year old woman with a history of recurrent syncope over 3 years who presented with repeated episodes of cardiac arrest due to bradycardia-dependent torsade de pointes (TdP), which continued long after possible triggers were eliminated. During these episodes a short coupling interval of the first tacycardia beat was also observed. The patientʹs stormy course was only controlled with pacing. She received a permanent dual-chamber pacemaker and prophylactically a beta-blocker was added. Over the subsequent 14 months she has remained completely asymptomatic.