[Prolonged Q-T interval with functional A-V block in neonates and young infants (author's transl)].
A prolonged Q-T interval (0.56 to 0.70 s.) with second degree A-V block was present in 5 children. Auscultation in utero (2 cases) or at birth (1 case) and syncopal attacks at 15 days and 2 months of life led to the discovery of the arrhythmia which was complicated with torsades de pointes. One patient died at 1 month of age; another is treated with propranolol and an internal pacemaker; the last 3 recovered, either spontaneously or with propranolol. The originality of this syndrome of unknown etiology is due to the A-V block, secondary to delayed repolarization (prolonged Q-T interval). Torsades de pointes and ventricular fibrillation are the main risks. Treatment includes beta adrenergic blocking agents associated with a temporarily implanted pacemaker. In a few cases, the internal pacemaker has to be left permanently.
D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D001145:Arrhythmias, Cardiac / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D002037:Bundle-Branch Block / Q000175:diagnosis; D002304:Cardiac Pacing, Artificial; D004562:Electrocardiography; D005260:Female; D006327:Heart Block / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D013575:Syncope / Q000209:etiology
D000319:Adrenergic beta-Antagonists
Allongement de l'espace QT avec bloc auriculo-ventriculaire fonctionnel chez le nouveau-né et le jeune nourrisson.