Catecholaminergic Polymorphic Ventricular Tachycardia: Difference between revisions
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Catecholaminergic Polymorphic Ventricular Tachycardia (view source)
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[[File: | [[File:ECG000032.jpg|thumb|300px|right|The ECG of a patient with CPVT in rest is normal]] | ||
[[File: | [[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. | ||
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'''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'''<cite> | '''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=== | ||
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#ACC2006 pmid=16935995 | #ACC2006 pmid=16935995 | ||
#sumitomo pmid=12482795 | #sumitomo pmid=12482795 | ||
#Wilde pmid=18463378 | |||
#Collura pmid=19467503 | |||
</biblio> | </biblio> | ||