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Catecholaminergic Polymorphic Ventricular Tachycardia: Difference between revisions
Catecholaminergic Polymorphic Ventricular Tachycardia (view source)
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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]], 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) | ||
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'''Treatment'''<cite>ACC2006</cite> | '''Treatment'''<cite>ACC2006</cite> | ||
* Beta-blockers | * Beta-blockers | ||
* [[w:nl:Internal_Cardiac_Defibrillator|ICD]] implantation combined with beta-blockers in CPVT patients who survived a cardiac arrest.<cite>sumitomo</cite> | * [[w:nl:Internal_Cardiac_Defibrillator|ICD]] 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>sumitomo</cite> | ||
* Surgical left cardiac sympathetic denervation in selected patients whose symptoms and/or ventricular arrhythmias are not controlled by pharmacologic therapy (Wilde, NEJM, 2009; Collura, Heart Rhythm, 2009) | |||
* Avoid competitive and other strenuous exercise | * Avoid competitive and other strenuous exercise | ||