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 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.

Articles of Interest

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?

Resources

British Heart Foundation

CPVT is a rare inherited heart rhythm disturbance. It usually happens when someone is under physical or emotional stress and can trigger an abnormally fast heartbeat. CPVT often first appears in childhood and in young people. Roughly 1 in 3 people need to be fitted with an ICD if medication is not enough to reduce the risk of an dangerous abnormal heart rhythm.

GeneReviews

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by episodic syncope occurring during exercise or acute emotion. The underlying cause of these episodes is the onset of fast ventricular tachycardia (bidirectional or polymorphic). Spontaneous recovery may occur when these arrhythmias self-terminate. In other instances, ventricular tachycardia may degenerate into ventricular fibrillation and cause sudden death if cardiopulmonary resuscitation is not readily available.