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| Catecholaminergic polymorphic ventricular tachycardia Classification and external resources |
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| OMIM | 604772 611938 |
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| DiseasesDB | 33816 |
Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an inherited heart rhythm disorder caused by a mutation in voltage gated ion channels and resulting in arrhythmias.
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Presentation
CPVT may cause exercise-induced ventricular arrhythmias and/or syncope occurring during physical activity or acute emotion, but demonstrates no structural problems of the heart. Ventricular tachycardia may self-terminate or degenerate into ventricular fibrillation, causing sudden death without immediate cardiopulmonary resuscitation. The majority of events occur during childhood and more than 60% of affected individuals will have a first episode of syncope or cardiac arrest by age 20.
Inheritance
CPVT has an autosomal dominant inheritance pattern. There are two genes currently associated with CPVT: RYR2 (majority) and CASQ2 (1-2%).
- The Ryanodine receptor (RYR2) is involved in intracardiac Ca2+ handling; Ca2+ overload triggers abnormal cardiac activity. [1]
- Calsequestrin (CASQ2) is a calcium buffering protein of the sarcoplasmic reticulum.
Diagnosis
CPVT is diagnosis based on reproducing ventricular arrhythmias during exercise stress testing, syncope occurring during physical activity and acute emotion, and a history of exercise or emotion-related palpitations and dizziness with an absence of structural cardiac abnormalities. The resting electrocardiogram is usually unremarkable but can show sinus bradycardia and a prominent "U". [2]
Genetic testing is sometimes available,[3] and is particularly useful for presymptomatic diagnosis of related individuals.
Treatment
CPVT is treated with beta blockers, verapamil or an implantable cardioverter-defibrillator.
References
- ^ Wehrens XH, Marks AR (November 2004). "Sudden unexplained death caused by cardiac ryanodine receptor (RyR2) mutations". Mayo Clin. Proc. 79 (11): 1367–71. PMID 15544013.
- ^ Aizawa Y, Komura S, Okada S, et al (May 2006). "Distinct U wave changes in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT)" (dead link – Scholar search). Int Heart J 47 (3): 381–9. doi:. PMID 16823244.
- ^ Genetic Testing: How to Navigate the Maze - SADS Foundation
Further reading
- Receptor defects cause inherited disorder CPVT
- Denervation successfully treats catecholaminergic polymorphic ventricular tachycardia
- Screening relatives of sudden-death victims provides likely cause of death and potentially saves lives
- Oxford Journals - European Heart Journal, Volume 18, Number 3, Pp. 530-531
- Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Information sheet - Auckland District Health Board's Cardiac Inherited Disease Registry
- Clinical Data's PGxHealth Division Launches CPVT Cardiac Channelopathy Test - Business Wire
- SADS UK - What is CPVT
- Arrhythmogenesis in CPVT: Lessons Learned from a CPVT Mouse Model
See also
External links
- Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Information Sheet
- The Hannah Wernke Memorial Foundation
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Wikipedia content modification information:
- This page was last modified on 6 July 2008, at 13:18.
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