Catecholaminergic polymorphic ventricular tachycardia overview: Difference between revisions
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==Historical Perspective== | ==Historical Perspective== | ||
Catecholaminergic polymorphic ventricular tachycardia ([[CPVT]]) was first described by [[Reid et al]] in 1975. It was described as a familial [[cardiac arrhythmia]] that occurs in patients with structurally normal [[heart]] and causes [[exercise]] or emotion triggered [[syncope]] and [[sudden death]] with a distinguishing pattern of [[ventricular arrhythmias|ventricular]] and [[supraventricular arrhythmias]]. In 2001, cardiac [[ryanodine receptor 2|Ryanodine Receptor]] gene ([[ryanodine receptor 2|RyR2]]) [[mutations]] were first implicated in the pathogenesis of catecholaminergic polymorphic ventricular tachycardia ([[CPVT]]). Subsequent experimental studies demonstrated that the abnormal [[calcium]] release from the [[sarcoplasmic reticulum]] caused [[arrhythmias]] mediated by delayed afterdepolarizations and triggered activity. | |||
==[[Catecholaminergic polymorphic ventricular tachycardia classification|Classification]]== | ==[[Catecholaminergic polymorphic ventricular tachycardia classification|Classification]]== |
Revision as of 18:29, 22 July 2020
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Differentiating Catecholaminergic polymorphic ventricular tachycardia from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a rare inherited arrhythmogenic disorder characterized by syncopal attacks, ventricular arrhythmias, and even sudden cardiac death, mostly in young patients. It is caused by mutations in calcium handling proteins such as RyR2 and CASQ2 within the sarcoplasmic reticulum, which results in ventricular arrhythmias in the setting of a high adrenergic tone such as during physical exercise or strong emotions. There are no associated structural abnormalities of the heart.
Historical Perspective
Catecholaminergic polymorphic ventricular tachycardia (CPVT) was first described by Reid et al in 1975. It was described as a familial cardiac arrhythmia that occurs in patients with structurally normal heart and causes exercise or emotion triggered syncope and sudden death with a distinguishing pattern of ventricular and supraventricular arrhythmias. In 2001, cardiac Ryanodine Receptor gene (RyR2) mutations were first implicated in the pathogenesis of catecholaminergic polymorphic ventricular tachycardia (CPVT). Subsequent experimental studies demonstrated that the abnormal calcium release from the sarcoplasmic reticulum caused arrhythmias mediated by delayed afterdepolarizations and triggered activity.
Classification
Pathophysiology
Causes
Differentiating Catecholaminergic polymorphic ventricular tachycardia from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Interventions | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies