Idiopathic Ventricular Fibrillation in a 29-Year-Old Man

Abstract

A 29-year-old man with a recent history of cardiac arrest and placement of an implanted cardiac defibrillator (ICD) presented with palpitations and defibrillation shocks. Five months earlier, he had experienced ventricular fibrillation (VF) while seated at his desk job. After resuscitation, an extensive diagnostic evaluation for the etiology of his arrest was undertaken, including left heart catheterization with coronary angiography, a transthoracic echocardiogram, and cardiac magnetic resonance imaging. All of these studies were unremarkable, revealing no evidence of ischemic or structural cardiac disease. He was subsequently discharged after placement of a single-lead endocardial ICD.After discharge, the patient remained asymptomatic until this presentation, when he reported palpitations, light-headedness, and multiple device shocks while awake. These symptoms were coincident with the onset of a febrile, upper respiratory tract infection. Vital signs, physical examination, complete blood count, and electrolyte panel were normal. The patient's 12-lead ECG is shown in Figure 1. What is the most likely diagnosis for this patient, and what is the best course of treatment?Please turn the page to read the diagnosis.

Modeling polymorphic ventricular tachycardia at rest using patient-specific induced pluripotent stem cell-derived cardiomyocytes

“…It also does not parallel RyR1 hot-spot regions for malignant hyperthermia [ 32 ]. Recently, other PMVT and torsades de pointe-related mutations on RyR2 have been reported [ 33 , 34 ] and like RyR2-H29D, the RyR2 M995V mutation associated with short coupled PMVT also does not lie within the RyR2 hot-spot regions. Therefore, the atypical locations of the RyR2-H29D and RyR2 M995V mutations likely account for the unique phenotypic expression of short-coupled PMVT at rest instead of CPVT or ARVC [ 35 , 36 ].…”

Recurrent Ventricular Fibrillation in an Apparently Healthy Patient

“…However, neither verapamil nor radiofrequency can completely eliminate the risk of recurrent VF or VT. Therefore, ICD placement is still recommended for most patients with SC-PMVT/VF …”

Mode of initiation and clinical significance of malignant rapid ventricular arrhythmias

“…[28] Calcium channel blocking agents, such as verapamil, have demonstrated efficacy in short-coupled Torsades de pointes. [29,30] VF or PVT occurring in patients with secondary huge abnormal J wave and long Q-T interval is very serious, and it is difficult to maintain sinus rhythm. The key for the patients with secondary huge abnormal J wave is to treat the primary disease.…”