Catecholaminergic Polymorphic Ventricular Tachycardia: The Cardiac Arrest Where Epinephrine Is Contraindicated

Those involved in resuscitation of young people should be aware of catecholaminergic polymorphic ventricular tachycardia and be suspicious of persistent ventricular ectopy, polymorphic, or bidirectional ventricular tachycardia during resuscitation. Appropriate management is avoidance of epinephrine, administration of general anesthesia, IV opiates, and consideration of flecainide.

Catecholaminergic Polymorphic Ventricular Tachycardia

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterised by adenergically mediated bidirectional and/or polymorphic ventricular tachycardia. CPVT is a significant cause of autopsy-negative sudden death in children and adolescents, although it can also affect adults. It is often caused by pathogenic variants in the cardiac ryanodine receptor gene as well as other rarer genes. Early identification and risk stratification is of major importance. β-blockers are the cornerstone of therapy. Sodium channel blockers, specifically flecainide, have an additive role.

Classic Presentation of Catecholaminergic Polymorphic Ventricular Tachycardia: A Case Report

This condition is estimated to have a prevalence of one per 10,000 persons. CPVT is characterized by ventricular arrhythmias at elevated heart rates which present as syncope, palpitations, SCD, or SCA following an emotionally or psychologically stressful event.

Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Βlockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal.

ED Case of Catecholaminergic Polymorphic Ventricular Tachycardia

The mean age of onset is 7-12 years, so this is initially a pediatric problem. However, onset may be as late as 40 years. The first manifestation may be cardiac arrest. The baseline ECG is normal. There are multiple mechanisms involved, including re-entry and delayed afterdepolarizations due to calcium overload or changes in autonomic tone. It generally cannot be induced in the EP lab.

Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: A Translational Perspective for the Clinician-Scientist

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare and potentially lethal inherited arrhythmia disease characterized by exercise or emotion-induced bidirectional or polymorphic ventricular tachyarrhythmias. The median age of disease onset is reported to be approximately 10 years of age. The majority of CPVT patients have pathogenic variants in the gene encoding the cardiac ryanodine receptor, or calsequestrin.

The Athlete With Catecholaminergic Polymorphic Ventricular Tachycardia

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is uncommon but recognized as a cause of sudden cardiac death (SCD) in those with structurally normal hearts. It typically presents with palpitations or syncope during intense physical or emotional stress and may be fatal.

The Kill of the Thrill

Can you die from excitement?