Congenital complete heart block and long Q-T syndrome requiring ventricular pacing for control of refractory ventricular tachycardia and fibrillation.
A three-year-old girl with congenital complete heart block presented with repeated bouts of ventricular tachycardia and ventricular fibrillation. The ECG was remarkable for both complete heart block and a long Q-T interval, when corrected for rate. The Q-T interval was longer than the Q-T interval of children with congenital complete heart block and of children without heart disease. Overdrive ventricular pacing was necessary to control the arrhythmias. A prolonged Q-T interval in patients with complete heart block, even in the presence of a normal QRS duration, may predispose the patient to sudden death. Permanent pacing can suppress these arrhythmias by overdriving.
D002304:Cardiac Pacing, Artificial; D002675:Child, Preschool; D004562:Electrocardiography; D005260:Female; D006327:Heart Block / Q000151:congenital* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D013610:Tachycardia / Q000503:physiopathology / Q000628:therapy*; D014693:Ventricular Fibrillation / Q000503:physiopathology / Q000628:therapy*