Unexpected Pediatric Cardiac Arrest in a Toddler: A Case of Catecholaminergic Polymorphic Ventricular Tachycardia Presenting as Ventricular Fibrillation.
Abiezer Disla Cuevas, Chantel Tran, Mohamud Daya et al.
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Case in brief
A previously healthy 2-year-old boy became pulseless after collapsing during a tantrum. EMS found ventricular fibrillation and delivered a 70-J biphasic shock (~4 J/kg), restoring circulation. He later had episodes of seizure-like posturing and limb rigidity, while his baseline ECG showed sinus tachycardia and echocardiography found no structural abnormality. An epinephrine challenge provoked bidirectional ventricular tachycardia, supporting catecholaminergic polymorphic ventricular tachycardia (CPVT); genetic testing identified a likely pathogenic RYR2 variant. He was discharged on propranolol and flecainide, with a loaner AED and outpatient follow-up arranged.
What made this case unusual
The toddler’s CPVT presented as ventricular fibrillation arrest, with subsequent seizure-like episodes that could represent recurrent arrhythmia; the resting ECG and echocardiogram were unremarkable.
Diagnostic / clinical pearl
In a child resuscitated from ventricular fibrillation, seizure-like posturing may reflect recurrent arrhythmia. A normal baseline ECG and structurally normal heart do not exclude CPVT; in this case, epinephrine challenge provoked diagnostic bidirectional ventricular tachycardia.
Why it matters
The case underscores the importance of prompt prehospital rhythm recognition and defibrillation in pediatric cardiac arrest.