Paroxysmal familial ventricular fibrillation

Several syndromes have been described which include paroxysmal ventricular fibrillation during the early years of life. In the family presented here, three siblings had syncopal episodes and sudden death; a fourth had syncopal episodes and proved paroxysmal ventricular fibrillation. Important characteristics of the ECG were short P-R interval and prominent U wave. The episodes of fibrillation were induced by stressful emotional stimuli and could not be produced by exercise. Possible electrophysiologic mechanisms and an outline for approach to patients with this problem are presented. The patient has been successfully maintained without recurrent symptoms for years with propanolol therapy.