View source for Catecholaminergic Polymorphic Ventricular Tachycardia

{{authors|
|mainauthor= [[user:Drj|J.S.S.G. de Jong, MD]]
|advisor=
|coauthor= [[user:Pgpostema|P.G. Postema, MD]]
|moderator= [[user:Pgpostema|P.G. Postema, MD]]
|editor= 
}}
[[File:ECG000032.jpg|thumb|300px|right|The ECG of a patient with CPVT in rest is normal]]
[[File:ECG000033.jpg|thumb|300px|right|The ECG of the same patient with CPVT during exercise. Asterisks mark polymorphic ventricular beats.]]
'''Catecholaminergic Polymorphic Ventricular Tachycardia''' is a congenital disease that leads to exercise induced [[Ventricular Arrhythmias|ventricular arrhythmias]] and / or syncope and carries an increased risk of sudden death. 

'''Characteristics of CPVT:'''
*The mean onset of arrhythmias is 7-9 years
*Absence of structural cardiac abnormalities
*Normal resting ECG
*Syncope during physical activity or emotional stress

'''Diagnosis'''
*The diagnosis is based on the patient's clinical history (dizziness or syncope induced by exercise or emotional stress and a family history containing syncope or sudden death in young relatives related to similar triggers) and reproducible ventricular arrhythmias during [[Exercise Testing|exercise testing]]. The complexity of these arrhythmias often increases with increasing work load, starting with [[Ventricular Premature Beats]], and ending with bidirectional [[Ventricular Tachycardia|ventricular tachycardia]] to [[Ventricular Tachycardia|polymorphic ventricular tachycardia]]. 
*Two genes have been linked to CPVT. Both lead to a defect in intracellular calcium metabolism:
** the hRyR2 gene, coding for the cardiac ryanodine receptor: ([[w:OMIM|OMIM&trade;]] link {{OMIM2|180902}}) (50-55 % of patients)
** the CASQ2 gene, coding for the calsequestrine protein: ([[w:OMIM|OMIM&trade;]] link {{OMIM2|114251}}) (1-2 % of patients)

'''Treatment'''<cite>sumitomo</cite>
* Beta-blockers
* [[ICD|ICD (Internal Cardioverter Defibrillator)]] implantation combined with beta-blockers in CPVT patients who survived a cardiac arrest or patients with syncope and/or documented sustained [[Ventricular Tachycardia|ventricular tachycardia]] despite beta-blocker therapy.<cite>ACC2006</cite> 
* Surgical left cardiac sympathetic denervation in selected patients whose symptoms and/or ventricular arrhythmias are not controlled by pharmacologic therapy <cite>Wilde</cite><cite>Collura</cite>
* Avoid competitive and other strenuous exercise

===References===
<biblio>
#ACC2006 pmid=16935995
#sumitomo pmid=12482795
#Wilde pmid=18463378
#Collura pmid=19467503
</biblio>