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

Abstract

Objectives: To raise awareness among pediatric intensive care specialists of catecholaminergic polymorphic ventricular tachycardia; an uncommon cause of polymorphic ventricular tachycardia and ventricular fibrillation arrest in children and young adults where epinephrine (adrenaline), even when given according to international protocols, can be counter-productive and life-threatening. We review three cases of cardiac arrest in children, later proven to be catecholaminergic polymorphic ventricular tachycardia related, where delay in recognition of this condition resulted in significantly longer resuscitation efforts, more interventions, and a longer time to return of spontaneous circulation. Design: Retrospective case series. Setting: Tertiary children’s hospital. Patients and Results: Three previously well children 4, 5, and 10 years old presented with cardiac arrest triggered by light activity, partial water immersion, and running, respectively. Initial resuscitation was bystander cardiopulmonary resuscitation and community defibrillation in all three cases. Electrocardiograms revealed multifocal ventricular ectopy, and in two (4 and 10 yr old), this correlated with repeated administration of epinephrine during repeated ventricular tachycardia and ventricular fibrillation cardiac arrest resuscitation cycles. This ultimately resolved immediately (at 78 and 140 min, respectively) with IV opiates once catecholaminergic polymorphic ventricular tachycardia was suspected. During recovery, on extracorporeal membrane oxygenation, epinephrine challenge in two children induced polymorphic ventricular tachycardia, bidirectional ventricular tachycardia, and ventricular fibrillation, which was cardioverted with flecainide in the 4-year-old. The third case was recognized early as catecholaminergic polymorphic ventricular tachycardia and was managed by avoiding epinephrine and using opiates and general anesthesia after the initial (single) cardioversion, and had a much better clinical course, without recourse to extracorporeal membrane oxygenation. All three carried de novo RyR2 (cardiac ryanodine) mutations. Conclusions: 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.

RYR2 receptor gene mutation associated with catecholaminergic polymorphic ventricular tachycardia in children: a case report & literature review

“…Furthermore, the same study also reported in nine patients who had positive respond to adrenaline or epinephrine challenge test after inducing VT. Similarly in a case series reported by Bellamy et al , they also demonstrated in three different patients (age range, 4–10 years old), a positive response to epinephrine challenge test by inducing arrythmias in patients with RYR2 gene mutation, which successfully reverted by nadolol and flecainide ( 43 ).…”

Cardiac Arrest From Undiagnosed Catecholaminergic Polymorphic Ventricular Tachycardia: A Case Report

“…In the differential diagnosis of sudden cardiac arrest without structural heart abnormalities, CPVT should always be considered along with similar conditions such as long QT syndrome, Brugada syndrome, and early repolarization syndrome [ 8 ]. Clinicians should hold a high index of suspicion for CPVT if the following features are exhibited: (i) cardiac arrest occurred during strong emotion or exercise; (ii) patient was previously healthy; (iii) EKG is normal at rest; (iv) adrenergic stimulation induces ventricular ectopy; and (v) ectopic beats become less frequent with anesthesia and opiates.…”

“…Clinicians should hold a high index of suspicion for CPVT if the following features are exhibited: (i) cardiac arrest occurred during strong emotion or exercise; (ii) patient was previously healthy; (iii) EKG is normal at rest; (iv) adrenergic stimulation induces ventricular ectopy; and (v) ectopic beats become less frequent with anesthesia and opiates. Furthermore, the finding of bidirectional ventricular tachycardia is pathognomonic for CPVT [ 8 ]. The clinical diagnosis of CPVT is dependent on stress-induced symptoms, family history, catecholamine infusion, and exercise stress testing.…”

Management of patients with an electrical storm or clustered ventricular arrhythmias: a clinical consensus statement of the European Heart Rhythm Association of the ESC—endorsed by the Asia-Pacific Heart Rhythm Society, Heart Rhythm Society, and Latin-American Heart Rhythm Society

“…Beta-blockers remain the first line of therapy, and epinephrine should be avoided, including in the setting of cardiac arrest/resuscitation. 174 …”