Catecholaminergic polymorphic ventricular tachycardia

Catecholaminergic polymorphic ventricular tachycardia

citationstyle = July 2008
expert = Medicine
expand = July 2008
update = July 2008
technical = July 2008

Caption =
DiseasesDB = 33816
ICD10 =
ICD9 =
OMIM = 604772
OMIM_mult = OMIM2|611938 | MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID =

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an inherited heart rhythm disorder caused by a mutation in voltage gated ion channels and resulting in arrhythmias.


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.


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. [cite journal |author=Wehrens XH, Marks AR |title=Sudden unexplained death caused by cardiac ryanodine receptor (RyR2) mutations |journal=Mayo Clin. Proc. |volume=79 |issue=11 |pages=1367–71 |year=2004 |month=November |pmid=15544013 |doi= |url= ]
* Calsequestrin (CASQ2) is a calcium buffering protein of the sarcoplasmic reticulum.


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". [cite journal |author=Aizawa Y, Komura S, Okada S, "et al" |title=Distinct U wave changes in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) |journal=Int Heart J |volume=47 |issue=3 |pages=381–9 |year=2006 |month=May |pmid=16823244 |doi=10.1536/ihj.47.381 |url= |format=dead link|date=July 2008 – [ Scholar search] ]

Genetic testing is sometimes available, [ [ Genetic Testing: How to Navigate the Maze - SADS Foundation] ] and is particularly useful for presymptomatic diagnosis of related individuals.


CPVT is treated with beta blockers, verapamil or an implantable cardioverter-defibrillator.


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]

ee also

* Brugada syndrome
* Cardiac arrhythmia
* Long QT syndrome
* Sick sinus syndrome
* Sudden cardiac death

External links

* [ Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Information Sheet]
* [ The Hannah Wernke Memorial Foundation]

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