Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology, Volume 21, Issue 10, 01 October 2019, Pages 1519-1526 Incidence and predictors of implantable cardioverter-defibrillator therapy and its complications in idiopathic ventricular fibrillation patients. Blom LJ, Visser M, Christiaans I, Scholten MF, Bootsma M, van den Berg MP, Yap SC, van der Heijden JF, Doevendans PA, Loh P, Postema PG, Barge-Schaapsveld DQ, Hofman N, Volders PGA, Wilde AA, Hassink RJ


Idiopathic ventricular fibrillation (IVF) is a rare cause of sudden cardiac arrest. Implantable cardioverter-defibrillator (ICD) implantation is currently the only treatment option. Limited data are available on the prevalence and complications of ICD therapy in these patients. We sought to investigate ICD therapy and its complications in patients with IVF.

Methods and results

Patients were selected from a national registry of IVF patients. Patients in whom no underlying diagnosis was found during follow-up were eligible for inclusion. Recurrence of ventricular arrhythmia (VA) was derived from medical and ICD records, electrogram records of ICD therapies were used to differentiate between appropriate or inappropriate interventions. Independent predictors for appropriate ICD shock were calculated using cox regression. In 217 IVF patients, recurrence of sustained VAs occurred in 66 patients (30%) during a median follow-up period of 6.1 years. Ten patients died (4.6%). Thirty-eight patients (17.5%) experienced inappropriate ICD therapy, and 32 patients (14.7%) had device-related complications. Symptoms before cardiac arrest [hazard ratio (HR)

2.51, 95% confidence interval (CI)

1.48-4.24], signs of conduction disease (


2.27, 95%


1.15-4.47), and carrier of the DPP6 risk haplotype (


3.24, 1.70-6.17) were identified as independent predictors of appropriate shock occurrence.


Implantable cardioverter-defibrillator therapy is an effective treatment in IVF, treating recurrences of potentially lethal VAs in approximately one-third of patients during long-term follow-up. However, device-related complications and inappropriate shocks were also frequent. We found significant predictors for appropriate ICD therapy. This may imply that these patients require additional management to prevent recurrent events.

Keywords: ventricular arrhythmia, Implantable Cardioverter-defibrillator, Idiopathic Ventricular Fibrillation, Implantable Cardioverter-defibrillator Therapy, Primary Electrical Disease

Europace. 2019 Oct;21(10):1519-1526