Document Detail

Guidelines for the diagnosis and management of Catecholaminergic Polymorphic Ventricular Tachycardia.
MedLine Citation:
PMID:  22119737     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an inherited arrhythmia syndrome, characterised by polymorphic ventricular tachycardia induced by adrenergic stress. CPVT can be caused by mutations the cardiac ryanodine receptor gene (RYR2) or mutations in the cardiac calsequestrin gene CASQ2. Structural heart disease is usually absent and the baseline ECG is usually normal. Patients with CPVT often present with exercise- or emotion induced syncope, the first presentation can also be sudden cardiac death.
MANAGEMENT: Besides removal of triggers treatment with beta blockers is currently a class I indication in clinically diagnosed patients. Beta blockage should be titrated up to an effective level. The addition of flecainide seems to be a promising approach in patients where arrhythmias are not completely suppressed by beta blockers. A cardioverter-defibrillator (ICD) or left cervical sympathetic denervation might be considered under special circumstances. Genetic counselling is recommended and all first degree relatives should be properly evaluated.
Andreas Pflaumer; Andrew M Davis
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Publication Detail:
Type:  Journal Article; Review     Date:  2011-11-25
Journal Detail:
Title:  Heart, lung & circulation     Volume:  21     ISSN:  1444-2892     ISO Abbreviation:  Heart Lung Circ     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-01-31     Completed Date:  2012-06-26     Revised Date:  2013-05-20    
Medline Journal Info:
Nlm Unique ID:  100963739     Medline TA:  Heart Lung Circ     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  96-100     Citation Subset:  IM    
Copyright Information:
Crown Copyright © 2011. Published by Elsevier B.V. All rights reserved.
The Royal Children's Hospital Melbourne, Australia.
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MeSH Terms
Adrenergic beta-Antagonists / therapeutic use*
DNA / genetics
Defibrillators, Implantable*
Diagnosis, Differential
Electrocardiography / methods*
Genetic Predisposition to Disease
Practice Guidelines as Topic*
Ryanodine Receptor Calcium Release Channel / genetics
Tachycardia, Ventricular* / diagnosis,  genetics,  therapy
Reg. No./Substance:
0/Adrenergic beta-Antagonists; 0/Ryanodine Receptor Calcium Release Channel; 9007-49-2/DNA

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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