Catecholaminergic Polymorphic Ventricular Tachycardia: Difference between revisions
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|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]] | ||
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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) | ||
Revision as of 13:11, 26 July 2007
 This article or section is currently being developed or reviewed. Some statements may be disputed, incorrect or biased. |
| Author(s) | J.S.S.G. de Jong, MD | |
| Moderator | P.G. Postema, MD | |
| Supervisor | ||
| some notes about authorship | ||
Catecholaminergic Polymorphic Ventricular Tachycardia is a congenital disease that leads to exercise induced ventricular arrhythmias and / or syncope and carries an increased risk of sudden death.
Characteristics of CPVT:
- Arrhythmias can start at young age, but sometimes do not occur before adulthood.
Diagnosis
- The diagnosis is based on reproducible ventricular arrhythmias during 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.
- Two genes have been linked to CPVT. Both lead to a defect in intracellular calcium metabolism:
Treatment Treatment consist of:ACC2006
- Beta-blockers
- ICD implantation combined with beta-blockers in CPVT patients who survived a cardiac arrest.
- Avoid competitive and other strenuous exercise
External Links
References
<biblio>
- ACC2006 pmid=16935995
</biblio>