Atrioventricular dissociation with prolonged QT interval and syncopal attacks in a 10-year-old boy.

Two heritable syndromes possibly genotypically related and manifesting prolonged QT (or QU) interval and TU wave changes, sinus bradycardia, syncopal attacks, ventricular arrhythmias, and frequently sudden death, are recognized respectively with (Jervell and Lange-Nielsen, 1957) and without (Romano, Gemme, and Pongiglione, I963; Ward, I964) profound childhood perceptive deafness. We report here a case seemingly of the latter syndrome but with atrioventricular dissociation as the dominant rhythm. Persistent supraventricular arrhythmias are only rarely documented in these syndromes (James, I967), and moreover the present case has other features that further warrant its report.