Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare channelopathy involving cardiac calcium metabolism that often shows up at an early age with misleading clinical symptoms, such as emotion- or exercise-related syncope with a normal resting electrocardiogram. In addition, it might be the underlying cause of sudden cardiac arrest in children or young adults. The particular pathophysiology of CPVT makes it particularly challenging for both resuscitation and the subsequent intensive care management after return of spontaneous circulation (ROSC).
We describe a case of sudden cardiac arrest in an 11-year-old girl affected by CPVT, with a particular focus on the most challenging aspects of resuscitation and intensive care management in light of other experiences found in the literature. A warning about the prodysrythmicity of mild hypothermia induced in the context of post-ROSC targeted temperature management in this particular population of patients and its possible physiopathological basis are discussed.
Why Should an Emergency Physician Be Aware of This?
CPVT is a rare but potentially lethal cause of stress-related syncope and sudden cardiac arrest in children and young adults. The diagnosis of CPVT requires a high level of suspicion and an interdisciplinary approach, including some adjustments during resuscitation and post–cardiac arrest care.
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Published online: March 20, 2020
Accepted: January 12, 2020
Received in revised form: November 28, 2019
Received: October 4, 2019
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