Catecholaminergic polymorphic ventricular tachycardia, an update.
Andrés Ricardo Pérez RieraRaimundo Barbosa-BarrosMarianne Penachini da Costa de Rezende BarbosaRodrigo Daminello-RaimundoAugusto A de LuccaLuiz C de AbreuPublished in: Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc (2017)
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.
Keyphrases
- emergency department
- cardiac arrest
- end stage renal disease
- left ventricular
- ejection fraction
- newly diagnosed
- genome wide
- chronic kidney disease
- healthcare
- young adults
- liver failure
- atrial fibrillation
- peritoneal dialysis
- cardiopulmonary resuscitation
- heart failure
- attention deficit hyperactivity disorder
- prognostic factors
- physical activity
- copy number
- gene expression
- high intensity
- low dose
- dna methylation
- catheter ablation
- mitral valve
- late onset
- drug induced
- patient reported outcomes
- early onset
- patient reported
- free survival
- heat stress