Torsade de pointes and asystole in a child with complete heart block and prolonged QT interval

Three-year-old girl with eight month history of convulsive syncope due to complete heart block, prolonged QT interval and paroxysms of ventricular tachycardia is described. An episode of torsade de pointes tachycardia followed by asystole was documented. The symptoms and electrocardiographic abnormalities were controlled by pacemaker therapy. The subset of "congenital heart block" patients with prolonged QT interval is at high risk of sudden death and should be regarded as a previously unrecognized form of Romano-Ward syndrome.