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 Vol. 23; no. 4; pp. 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:	Journal Article
Language:	English
Published:	United States John Wiley & Sons, Inc 01-07-2018 John Wiley and Sons Inc
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 normal structural heart catecholaminergic polymorphic ventricular tachycardia sudden cardiac death/arrest channelopathies
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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.
Bibliography:	ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-3 content type line 23 ObjectType-Review-1
ISSN:	1082-720X 1542-474X
DOI:	10.1111/anec.12512