Down, Not Out: eCPR for Refractory Ventricular Tachyarrhythmia in a Child With Brugada Syndrome

Introduction: Children with refractory ventricular tachyarrhythmia (VT) represent a minority of pediatric cardiac arrests with prolonged conventional cardiopulmonary resuscitation (cCPR). Extracorporeal CPR (eCPR) is a rescue therapy when spontaneous circulation is not achieved with cCPR. Although Brugada Syndrome is a known etiology of VT, the use of eCPR in refractory VT from Brugada Syndrome is not well characterized in pediatric literature. Here, we present the second such described use of pediatric eCPR.


Description: A 7 year-old previously healthy male presents with nocturnal episodes of abnormal breathing and movement. Although initially vitally stable with a reassuring exam, telemetry demonstrates self-resolving runs of polymorphic VT, ultimately progressing to pulseless cardiac arrest. Over the initial hour of resuscitation, he receives 4 defibrillations, epinephrine, amiodarone, magnesium, and calcium for refractory VT transiently converting to normal sinus rhythm.

eCPR activation is sought for ongoing VT. In the intensive care unit, he demonstrates right-sided ST changes with bradycardia and normal QTc, and receives 9 additional defibrillations and isoproterenol for suspected Brugada Syndrome. Following peripheral cannulation to VA ECMO, he demonstrates immediate cessation of VT.

The first 48 hours post-arrest are absent of VT recurrence, including isoproterenol wean. He requires aggressive thermoregulation via ECMO circuit and external cooling devices to maintain normothermia. Temperatures normalize 120 hours post-arrest, after which a Procainamide challenge is pursued without provocation of diagnostic ECG findings. Following heavy sedation during a subsequent clamp trial on ECMO day 6, he develops characteristic Brugada Type I ECG changes with bradycardia and is initiated on quinidine prior to ECMO decannulation 24 hours later.


Discussion: Brugada Syndrome is a channelopathy with characteristic clinical features including stereotyped ECG abnormalities and classic environmental triggers (e.g. excess vagal tone, fevers, antiarrhythmics). In this case, the use of pediatric eCPR served to acutely manage cardiac arrest secondary to refractory VT and as a bridge to definitive diagnosis of Brugada Syndrome. Relevant diagnostic and management features for the intensivist team will be reviewed.