QT prolongation and syncopal attacks. A case of the Romano-Ward syndrome

The Romano-Ward syndrome is very rare as hereditary disease. Only 5 families have been reported in Japan before the present report. The patient, a 15-year-old female, had been treated for epilepsy due to syncopal attacks. Electrocardiography revealed occurrence of an augmented and labile U wave and prolongation of the Q-T interval, with recordings of frequent multifocal extrasystoles and transient ventricular fibrillation. Also noticed was sinus bradycardia. Clinical examinations presented no results coinciding with those specific ECG findings. It was inferred through surveys of her family tree that the present case had an autosomal dominant trait, heterozygously affected through the male line. Severe arrhythmias detected on her ECGs were suppressed by oral administration of 30 mg/day propranolol. Judging from the fact, propranolol may be the first to be administered among a variety of anti-arrhythmic agents in the Romano-Ward syndrome.