Catecholaminergic Polymorphic Ventricular Tachycardia: Difference between revisions

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{{authors|
{{authors|
|mainauthor= [[user:Pgpostema|P.G. Postema, MD]]
|mainauthor= [[user:Drj|J.S.S.G. de Jong, MD]]
|advisor=
|advisor=
|coauthor= [[user:Drj|J.S.S.G. de Jong, MD]]
|coauthor= [[user:Pgpostema|P.G. Postema, MD]]
|moderator= [[user:Pgpostema|P.G. Postema, MD]]
|moderator= [[user:Pgpostema|P.G. Postema, MD]]
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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)