Catecholaminergic polymorphic ventricular tachycardia, an update

Catecholaminergic polymorphic ventricular tachycardia is a rare devastating lethal inherited disorder or sporadic cardiac ion channelopathy characterized by unexplained syncopal episodes, and/or sudden cardiac death (SCD), aborted SCD (ASCD), or sudden cardiac arrest (SCA) observed in children, adol...

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Bibliographic Details
Published in:Annals of noninvasive electrocardiology 2018-07, Vol.23 (4), p.e12512-n/a
Main Authors: Pérez‐Riera, Andrés R., Barbosa‐Barros, Raimundo, de Rezende Barbosa, Marianne P. C., Daminello‐Raimundo, Rodrigo, de Lucca, Augusto A., de Abreu, Luiz C.
Format: Article
Language:English
Subjects:
Adolescent
Adolescents
Adults
Arrhythmia
Cardiac arrhythmia
catecholaminergic polymorphic ventricular tachycardia
channelopathies
Channelopathy
Child
Child, Preschool
Children
Coronary artery disease
Defibrillators
Diagnosis
Differential diagnosis
EKG
Electrocardiography
Electrocardiography - methods
Emergency medical services
Epidemiology
Familiarity
Heart
Heart diseases
Heart Ventricles - diagnostic imaging
Heart Ventricles - physiopathology
Humans
normal structural heart
Review
Risk management
sudden cardiac death/arrest
Tachycardia
Tachycardia, Ventricular - diagnosis
Tachycardia, Ventricular - genetics
Tachycardia, Ventricular - physiopathology
Ventricle
Young Adult
Young adults
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Description
Summary:Catecholaminergic polymorphic ventricular tachycardia is a rare devastating lethal inherited disorder or sporadic cardiac ion channelopathy characterized by unexplained syncopal episodes, and/or sudden cardiac death (SCD), aborted SCD (ASCD), or sudden cardiac arrest (SCA) observed in children, adolescents, and young adults without structural heart disease, consequence of adrenergically mediated arrhythmias: exercise‐induced, by acute emotional stress, atrial pacing, or β‐stimulant infusion, even when the electrocardiogram is normal. The entity is difficult to diagnose in the emergency department, given the range of presentations; thus, a familiarity with and high index of suspicion for this pathology are crucial. Furthermore, recognition of the characteristic findings and knowledge of the management of symptomatic patients are necessary, given the risk of arrhythmia recurrence and SCA. In this review, we will discuss the concept, epidemiology, genetic background, genetic subtypes, clinical presentation, electrocardiographic features, diagnosis criteria, differential diagnosis, and management.
ISSN:1082-720X
1542-474X
DOI:10.1111/anec.12512