Catecholaminergic Polymorphic Ventricular Tachycardia: Difference between revisions
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Catecholaminergic Polymorphic Ventricular Tachycardia (view source)
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{{authors| | {{authors| | ||
|mainauthor= [[user: | |mainauthor= [[user:Drj|J.S.S.G. de Jong, MD]] | ||
|advisor= | |advisor= | ||
|coauthor= [[user: | |coauthor= [[user:Pgpostema|P.G. Postema, MD]] | ||
|moderator= [[user:Pgpostema|P.G. Postema, MD]] | |moderator= [[user:Pgpostema|P.G. Postema, MD]] | ||
|editor= | |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. | '''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:''' | '''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''' | '''Diagnosis''' | ||
*The diagnosis is based on reproducible ventricular arrhythmias during [[Exercise Testing|exercise testing]] | *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: | *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™]] link {{OMIM2|180902}}) (50-55 % of patients) | ** the hRyR2 gene, coding for the cardiac ryanodine receptor: ([[w:OMIM|OMIM™]] link {{OMIM2|180902}}) (50-55 % of patients) | ||
** the CASQ2 gene, coding for the calsequestrine protein: ([[w:OMIM|OMIM™]] link {{OMIM2|114251}}) (1-2 % of patients) | ** the CASQ2 gene, coding for the calsequestrine protein: ([[w:OMIM|OMIM™]] link {{OMIM2|114251}}) (1-2 % of patients) | ||
'''Treatment''' | '''Treatment'''<cite>sumitomo</cite> | ||
* Beta-blockers | * 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 | * Avoid competitive and other strenuous exercise | ||
===References=== | ===References=== | ||
<biblio> | <biblio> | ||
#ACC2006 pmid=16935995 | #ACC2006 pmid=16935995 | ||
#sumitomo pmid=12482795 | |||
#Wilde pmid=18463378 | |||
#Collura pmid=19467503 | |||
</biblio> | </biblio> | ||