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{{Catecholaminergic polymorphic ventricular tachycardia}}
'''For patient information, click [[Catecholaminergic polymorphic ventricular tachycardia(patient information)|here]].'''<br>


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{{CMG}}; {{AE}} {{MRV}}


{{SK}} CPVT, catecholaminergic polymorphic VT, bidirectional ventricular tachycardia induced by catecholamines, bidirectional VT, catecholamine-induced polymorphic ventricular tachycardia, catecholamine induced polymorphic ventricular tachycardia, familial polymorphic ventricular tachycardia, FPVT, polymorphic ventricular tachycardia, polymorphic VT induced by catecholamines.


==[[Catecholaminergic polymorphic ventricular tachycardia overview|Overview]]==


'''Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)''' is an inherited heart rhythm disorder caused by a mutation in voltage gated ion channels and resulting in arrhythmias.  CPVT may cause exercise-induced ventricular arrhythmias and/or syncope occurring during physical activity or acute emotion, but demonstrates no structural problems of the heart. Ventricular tachycardia may self-terminate or degenerate into ventricular fibrillation, causing sudden death without immediate cardiopulmonary resuscitation. The majority of events occur during childhood and more than 60% of affected individuals will have a first episode of syncope or cardiac arrest by age 20. 
==[[Catecholaminergic polymorphic ventricular tachycardia historical perspective|Historical Perspective]]==


==Inheritance==
==[[Catecholaminergic polymorphic ventricular tachycardia classification|Classification]]==


CPVT has an autosomal dominant inheritance pattern.  There are two genes currently associated with CPVT: RYR2 (majority) and CASQ2 (1-2%).  The Ryanodine receptor (RYR2) is involved in intracardiac Ca2+ handling; Ca2+ overload triggers abnormal cardiac activity.  Calsequestrin (CASQ2) is a calcium buffering protein of the sarcoplasmic reticulum.
==[[Catecholaminergic polymorphic ventricular tachycardia pathophysiology|Pathophysiology]]==
 
==[[Catecholaminergic polymorphic ventricular tachycardia causes|Causes]]==
 
==[[Catecholaminergic polymorphic ventricular tachycardia differential diagnosis|Differentiating Catecholaminergic polymorphic ventricular tachycardia from other Diseases]]==
 
==[[Catecholaminergic polymorphic ventricular tachycardia epidemiology and demographics|Epidemiology and Demographics]]==
 
==[[Catecholaminergic polymorphic ventricular tachycardia risk factors|Risk Factors]]==
 
==[[Catecholaminergic polymorphic ventricular tachycardia screening|Screening]]==
 
==[[Catecholaminergic polymorphic ventricular tachycardia natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


==Diagnosis==
==Diagnosis==
 
[[Catecholaminergic polymorphic ventricular tachycardia diagnostic study of choice|Diagnostic study of choice]] | [[Catecholaminergic polymorphic ventricular tachycardia history and symptoms|History and Symptoms]] | [[Catecholaminergic polymorphic ventricular tachycardia physical examination|Physical Examination]] | [[Catecholaminergic polymorphic ventricular tachycardia laboratory findings|Laboratory Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia electrocardiogram|Electrocardiogram]] | [[Catecholaminergic polymorphic ventricular tachycardia exercise stress testing|Exercise Stress Testing]] | [[Catecholaminergic polymorphic ventricular tachycardia genetic testing|Genetic Testing]] | [[Catecholaminergic polymorphic ventricular tachycardia x ray|X-Ray Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Catecholaminergic polymorphic ventricular tachycardia CT scan|CT-Scan Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia MRI|MRI Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia other imaging findings|Other Imaging Findings]] | [[Catecholaminergic polymorphic ventricular tachycardia other diagnostic studies|Other Diagnostic Studies]]
CPVT is diagnosis based on reproducing ventricular arrhythmias during exercise stress testing, syncope occurring during physical activity and acute emotion, and a history of exercise or emotion-related palpitations and dizziness with an absence of structural cardiac abnormalities. The resting electrocardiogram is usually unremarkable but can show sinus bradycardia and a prominent "U".  Genetic testing is sometimes available and is particularly useful for presymptomatic diagnosis of related individuals.


==Treatment==
==Treatment==
[[Catecholaminergic polymorphic ventricular tachycardia medical therapy|Medical Therapy]] | [[Catecholaminergic polymorphic ventricular tachycardia implantable cardioverter-defibrillator|Implantable Cardioverter-Defibrillator]] | [[Catecholaminergic polymorphic ventricular tachycardia surgery|Surgery]] | [[Catecholaminergic polymorphic ventricular tachycardia primary prevention|Primary Prevention]] | [[Catecholaminergic polymorphic ventricular tachycardia secondary prevention|Secondary Prevention]] | [[Catecholaminergic polymorphic ventricular tachycardia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Catecholaminergic polymorphic ventricular tachycardia future or investigational therapies|Future or Investigational Therapies]]


CPVT is treated with [[beta blockers]], [[verapamil]] or an [[Implantable cardioverter-defibrillator|ICD]] (implantable cardiac defibrillator).
==Case Studies==
[[Catecholaminergic polymorphic ventricular tachycardia case study one|Case #1]]


{{electrocardiography}}
==Related Chapters==
*[[Ventricular tachycardia]]
*[[Ventricular fibrillation]]
*[[Long QT syndrome]]
*[[Polymorphic ventricular tachycardia]]


[[Category:Cardiology]]
[[Category:Cardiology]]
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Latest revision as of 10:12, 24 July 2020

Catecholaminergic polymorphic ventricular tachycardia Microchapters

Home

Patient Information

Overview

Historical Perspective

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

Exercise Stress Testing

Genetic Testing

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Implantable Cardioverter-Defibrillator

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Catecholaminergic polymorphic ventricular tachycardia On the Web

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mounika Reddy Vadiyala, M.B.B.S.[2]

Synonyms and keywords: CPVT, catecholaminergic polymorphic VT, bidirectional ventricular tachycardia induced by catecholamines, bidirectional VT, catecholamine-induced polymorphic ventricular tachycardia, catecholamine induced polymorphic ventricular tachycardia, familial polymorphic ventricular tachycardia, FPVT, polymorphic ventricular tachycardia, polymorphic VT induced by catecholamines.

Overview

Historical Perspective

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 | Exercise Stress Testing | Genetic Testing | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Implantable Cardioverter-Defibrillator | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters