LONG QT SYNDROME MANIFESTED AS FETAL VENTRICULAR-TACHYCARDIA AND INTERMITTENT AV BLOCK

A case of long QT syndrome diagnosed in the early neonatal period is d escribed. This full-term female baby had intermittent atrioventricular (AV) block and ventricular tachycardia detected antenatally at the ge stational age of 26 weeks. Sinus rhythm with prolonged QT interval (QT c = 0.636 sec) was found soon after birth. She developed variable degr ee of AV block with alternating left and right bundle branch block, wh ich suggested the presence of multilevel AV block. Her mother had no l upus autoantibodies. Auditory brain stem evoked potential was normal. Family study revealed QT prolongation in her grandmother. Her conditio n improved after pacemaker implantation and oral beta-blocker usage.