Catecholaminergic Polymorphic Ventricular Tachycardia: Difference between revisions

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Catecholaminerge Polymorfe [[Ritmestoornissen#Ventrikeltachycardie|Ventriculaire Tachycardie]] is een erfelijke aandoening, die zich uit als inspanningsgeboden of door stress uitgelokte ventriculaire ritmestoornissen, [[syncope]] of plotse dood.  
'''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.  


De ritmestoornissen treden meestal al in de kinderleeftijd op, maar kunnen zich ook pas in de volwassen leeftijd manifesteren.
'''Characteristics of CPVT:'''
*Arrhythmias can start at young age, but sometimes do not occur before adulthood.  


De ventriculaire ritmestoornissen treden alleen op in situaties waarbij [[:w:catecholamine|catecholamines]] (zoals adrenaline) vrijkomen zoals fysieke inspanning en / of bij emotionele stress.
'''Diagnosis'''
In rust zijn er aan het ECG geen afwijkingen te zien.
*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.
Er zijn afwijkingen op 2 genen bekend die CPVT kunnen veroorzaken (een gen dat codeert voor de cardiale ryanodine receptor hRyR2 ([[w:OMIM|OMIM™]] link {{OMIM2|180902}}) en een gen dat codeert vor het eiwit calsequestrine CASQ2 ([[w:OMIM|OMIM™]] link {{OMIM2|114251}}). Beide genen spelen een rol in de calciumhuishouding van hartcellen. De calciumconcentratie in de cellen is daardoor verhoogd.
*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)


De prevalentie van CPVT is nog onbekend. De man/vrouw verhouding is 1.
'''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


===Behandeling===
===External Links===
De behandeling:<cite>ACC2006</cite>
* Beta-blokkers
* [[w:nl:Internal_Cardiac_Defibrillator|ICD]] implantatie in combinatie met beta-blokkers bij patiënten die een hartstilstand hebben overleefd
 
===Externe Links===
*[http://www.cardiogenetica.nl Cardiogenetica.nl]
*[http://www.cardiogenetica.nl Cardiogenetica.nl]
*[http://genetests.org/profiles/cvt/index.html GeneReviews CPVT]


===Referenties===
===References===
<biblio>
<biblio>
#ACC2006 pmid=16935995
#ACC2006 pmid=16935995
</biblio>
</biblio>

Revision as of 13:10, 26 July 2007

Accuracy dispute This article or section is currently being developed or reviewed.
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:
    • the hRyR2 gene, coding for the cardiac ryanodine receptor: (OMIM™ link 180902) (50-55 % of patients)
    • the CASQ2 gene, coding for the calsequestrine protein: (OMIM™ link 114251) (1-2 % of patients)

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

  1. Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC Jr, Jacobs AK, Adams CD, Antman EM, Anderson JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B, Blanc JJ, Budaj A, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL, American College of Cardiology/American Heart Association Task Force, European Society of Cardiology Committee for Practice Guidelines, European Heart Rhythm Association, and Heart Rhythm Society. ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Circulation. 2006 Sep 5;114(10):e385-484. DOI:10.1161/CIRCULATIONAHA.106.178233 | PubMed ID:16935995 | HubMed [ACC2006]
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