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:[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
- 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 |