Catecholaminergic Polymorphic Ventricular Tachycardia: Difference between revisions
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'''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. | |||
'''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|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: | |||
** 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) | |||
'''Treatment''' | |||
Treatment consist of:<cite>ACC2006</cite> | |||
* Beta-blockers | |||
* [[w:nl:Internal_Cardiac_Defibrillator|ICD]] implantation combined with beta-blockers in CPVT patients who survived a cardiac arrest. | |||
* Avoid competitive and other strenuous exercise | |||
=== | ===External Links=== | ||
*[http://www.cardiogenetica.nl Cardiogenetica.nl] | *[http://www.cardiogenetica.nl Cardiogenetica.nl] | ||
*[http://genetests.org/profiles/cvt/index.html GeneReviews CPVT] | |||
=== | ===References=== | ||
<biblio> | <biblio> | ||
#ACC2006 pmid=16935995 | #ACC2006 pmid=16935995 | ||
</biblio> | </biblio> |
Revision as of 13:10, 26 July 2007
![]() Some statements may be disputed, incorrect or biased. |
Author(s) | P.G. Postema, 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:[1]
- 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
Error fetching PMID 16935995:
- Error fetching PMID 16935995: