A Case Report: A Diagnosis of Catecholaminergic Polymorphic Ventricular Tachycardia Made in a Pediatric Patient in the Emergency Department Identified from Delay in Post Intubation Sedation

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Authors: Kevin Bosnoyan, MD, Rodney Fullmer, DO, MBS, FACEP, FACOEP
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Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited arrhythmia syndrome that can present with life-threatening ventricular arrhythmias in the absence of structural heart disease.Recognition in the emergency setting is challenging, and missed diagnosis is often fatal.A 16-year-old male presented to the Emergency Department after an out-ofhospital cardiac arrest with ventricular fibrillation and achieved return of spontaneous circulation following defibrillation and resuscitation.The event occurred after prolonged fasting and physical exertion.Initial evaluation revealed no structural cardiac abnormalities.Following intubation, delayed post-intubation sedation led to agitation which coincided with the development of frequent premature ventricular contractions and episodes of non-sustained ventricular tachycardia on continuous cardiac monitoring.Escalation of sedation resulted in improvement of ventricular ectopy.Consultation with pediatric electrophysiology raised concern for CPVT, prompting initiation of beta-blocker therapy.After stabilization, the patient was safely transferred to a tertiary pediatric center, where genetic testing identified a pathogenic RYR2 variant, confirming a diagnosis of CPVT.This case highlights the importance of continuous cardiac monitoring and vigilance for evolving arrhythmias in pediatric patients following resuscitation.Delayed or inadequate sedation may exacerbate catecholamine-mediated ventricular arrhythmias and unmask underlying channelopathies.Early recognition, optimization of sedation, prompt beta-blockade, and multidisciplinary collaboration were critical in preventing further clinical deterioration and facilitating safe transfer of care.

Fields of Study & Subjects

Cardiac electrophysiology and arrhythmias Cardiac Arrhythmias and Treatments Cardiovascular Effects of Exercise

Publication details

Journal / source
Emergency Medicine Investigations
Volume
11
Issue
1
Publication date
31 January 2026