Idiopathic ventricular fibrillation: clinical, electrophysiologic characteristics and long-term outcomes

The long-term prognosis, including risks of arrhythmic recurrence of idiopathic ventricular fibrillation (VF), is uncertain; moreover, the role of electrophysiologic study in the diagnosis and guiding of antiarrhythmic drugs therapy for idiopathic VF remains controversial. The purpose of this study was to study the clinical features, electrophysiologic characteristics and long-term clinical outcomes of six consecutive patients (five males) who had at least one episode of aborted cardiac arrest (5 patients) or syncope (1 patients) with documentation of ventricular fibrillation (VF) in the absence of apparent heart disease. Idiopathic VF was diagnosed by exclusion. All patients underwent the electrophysiologic study including intravenous antiarrhythmic drug testing. Recurrences of VF after therapy and the long-term outcomes were assessed. The mean age at the first episode was 43+/-19 years (range from 16 to 63 years). All patients had sustained VF induced by double (3 patients) or triple (3 patients) ventricular extrastimuli at a paced cycle length of 400 or 500 ms from the right ventricular apex. Intravenous procainamide and/or mexiletine could suppress the reinduction of sustained VF in 4 (67%) of 6 patients. Recurrence of VF (documented VF attack, sudden cardiac arrest or syncope) was observed in 3 (100%) of 3 patients who received procainamide or mexiletine alone. Four patients (including 3 patients who experienced recurrence) received amiodarone alone or in combination with mexiletine, and these drugs could effectively prevent recurrence of VF. One patient with exercise-induced VF remained asymptomatic without any treatment during a follow-up period of 95 months. Another patient received an implantable cardioverter-defibrillator without concomitant antiarrhythmic drug therapy and had no discharge of electrical shock during 28 months of follow-up. During a mean follow-up period of 64+/-40 months (range from 28 to 128 months), all the patients were alive except patient No. 2 who died of acute hepatic failure. In conclusion, electrophysiologic study is a reliable diagnostic method, but it was of limited value in guiding antiarrhythmic drug therapy for preventing recurrence of idiopathic VF. Class I drug alone was associated with a high recurrence rate (100%) despite predictions that it would be effective by the electrophysiologic study. Amiodarone alone or in combination with mexiletine effectively prevented the recurrence of VF during the long-term follow-up along with a favourable outcome.