Abstract
Background
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).
Case Report
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.
Keywords
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References
- Sudden cardiac death in long QT syndrome (LQTS), Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT).Prog Cardiovasc Dis. 2019; 62: 227-234
- Impact of genetics on the clinical management of channelopathies.J Am Coll Cardiol. 2013; 62: 169-180
- Coronary angiography after cardiac arrest without ST-segment elevation.N Engl J Med. 2019; 380: 1397-1407
- Part 8: post-cardiac arrest care: 2015 American Heart Association guidelines update for cardiopulmonary resuscitation and emergency cardiovascular care.Circulation. 2015; 132: S465-S482
- Catecholaminergic polymorphic ventricular tachycardia.Curr Opin Cardiol. 2017; 32: 78-85
- Catecholaminergic polymorphic ventricular tachycardia: the cardiac arrest where epinephrine is contraindicated.Pediatr Crit Care Med. 2019; 20: 262-268
- Nadolol decreases the incidence and severity of ventricular arrhythmias during exercise stress testing compared with β1-selective β-blockers in patients with catecholaminergic polymorphic ventricular tachycardia.Hear Rhythm. 2016; 13: 433-440
- Writing Committee Members 2017 AHA/ACC/HRS Guideline for Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death a Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice.Circulation. 2018; 138: 272-391
- Science review: vasopressin and the cardiovascular system part 2–clinical physiology.Crit Care. 2004; 8: 15-23
- A rare cause of sudden cardiac arrest: catecholaminergic polymorphic ventricular tachycardia.Turk Pediatr Ars. 2018; 53: 124-128
- Arrhythmogenic delayed afterdepolarizations are promoted by severe hypothermia but not therapeutic hypothermia.Circ J. 2017; 82: 62-70
- Increased Ca2+ sensitivity of the ryanodine receptor mutant RyR2R4496C underlies catecholaminergic polymorphic ventricular tachycardia.Circ Res. 2009; 104: 201-209
- The catecholamine, cortisol, and hemodynamic responses to mild perioperative hypothermia: a randomized clinical trial.Anesthesiology. 1995; 82: 83-93
- Guidelines for the diagnosis and management of catecholaminergic polymorphic ventricular tachycardia.Heart Lung Circ. 2012; 21: 96-100
- Efficacy of flecainide in the treatment of catecholaminergic polymorphic ventricular tachycardia.JAMA Cardiol. 2017; 2: 759
- Clinical management of catecholaminergic polymorphic ventricular tachycardia the role of left cardiac sympathetic denervation.Circulation. 2015; 131: 2185-2193
- Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: a systematic review.Heart Rhythm. 2018; 15: 1791-1799
- Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest.Eur Heart J. 2019; 40: 2953-2961
Article info
Publication history
Published online: March 20, 2020
Accepted:
January 12,
2020
Received in revised form:
November 28,
2019
Received:
October 4,
2019
Identification
Copyright
© 2020 Elsevier Inc. All rights reserved.
