Abstract
Sudden cardiac death in the truly normal heart is an uncommon occurrence. The majority of patients without apparent structural heart disease who died suddenly do not actually have “normal” hearts. Idiopathic ventricular fibrillation (IVF) is an uncommon disease of unknown etiology that manifests as syncope, cardiac arrest or seizures caused by rapid polymorphic ventricular tachycardia (VT) or VF in the absence of structural heart disease or identifiable channelopathy. Usually during an arrhythmic storm, it is relatively easy to diagnose IVF in a cardiac arrest survivor when the onset of spontaneous polymorphic VT/VF can be recorded, and this shows initiation of polymorphic VT/VF by very short coupled ventricular ectopy. IVF is essentially a diagnosis by exclusion. However, typical clinical and electrophysiological characteristics present in some patients often allows for a positive diagnosis. Since the rate of recurrence of malignant ventricular arrhythmias in IVF is unacceptably high in the absence of therapy, once a diagnosis of IVF is made, some form of therapy is mandatory. Therapy may include ICD implantation, drug therapy, radiofrequency catheter ablation of the triggering focus or combinations of the above. This article will discuss the role of pharmacological treatment in the therapeutic management of IVF. Is there still a place for pharmacological treatment in IVF? Yes, there is still a place for pharmacological treatment in the therapeutic armamentarium of IVF in carefully selected, individually well documented patients.
Highlights
Idiopathic ventricular fibrillation (IVF) is a rare disorder encountered in only 3% to 9% of cases of out-of-hospital ventricular fibrillation (VF) not related to an acute coronary syndrome
The risks of sudden cardiac death (SCD) are higher in patients with structural heart disease, and the underlying pathophysiology for the majority of these deaths is due to coronary artery disease, SCD events occur in individuals with apparently normal hearts [1,2,3,4,5]
Considering that an implantable cardioverter-defibrillator (ICD) terminates episodes of IVF but does not prevent arrhythmias, patients who have frequent symptoms or device discharges from recurrent arrhythmias may benefit from adjunctive anti-arrhythmic drug therapy, usually beginning with empiric amiodarone
Summary
Idiopathic ventricular fibrillation (IVF) is a rare disorder encountered in only 3% to 9% of cases of out-of-hospital ventricular fibrillation (VF) not related to an acute coronary syndrome. In a small number of cases of survivors of sudden cardiac death (SCD), despite extensive clinical evaluation, no underlying structural heart disease can be found. The risks of SCD are higher in patients with structural heart disease, and the underlying pathophysiology for the majority of these deaths is due to coronary artery disease, SCD events occur in individuals with apparently normal hearts [1,2,3,4,5]. The differential diagnosis of IVF included the following arrhythmia-disorders: the long QT syndrome [7,8,9], the catecholamine sensitive polymorphic VT (CPVT) [10], and the syndrome of nocturnal sudden death of South East Asia [11].
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