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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= | ||
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'''Diagnosis''' | '''Diagnosis''' | ||
*The diagnosis is based on reproducible ventricular arrhythmias during [[Exercise Testing|exercise testing]]. Typically the onset of ventricular arrhythmias is at around 100-120 bpm. The complexity of these arrhythmias often increases with increasing work load, starting with [[Ventricular Premature Beats]], bidirectional ventricular tachycardia to polymorphic ventricular tachycardia. | *The diagnosis is based on reproducible ventricular arrhythmias during [[Exercise Testing|exercise testing]]. Typically the onset of ventricular arrhythmias is at around 100-120 bpm. 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) |