Catecholaminergic polymorphic ventricular tachycardia

Question Question

I have been diagnosed with CPVT. I've been off medication for a few years and have been playing sports regularly. I've never had any syncope. I just read that if untreated CPVT is highly lethal. And now I'm starting to care. I have some questions about this disease: Is there any case of remission? Is it curable? Are sports under treatment better than without treatment? Is there a CPVT specialist anywhere in the world? What is the most up-to-date article/research about this disease?

Answer Answer

The following information may help to address your question:

Can catecholaminergic polymorphic ventricular tachycardia go into remission?
Do all people with catecholaminergic polymorphic ventricular tachycardia require treatment?
Can people with catecholaminergic polymorphic ventricular tachycardia play sports?
How might catecholaminergic polymorphic ventricular tachycardia be treated?
Can catecholaminergic polymorphic ventricular tachycardia be cured?
Are there any research studies involving catecholaminergic polymorphic ventricular tachycardia?
How can I find catecholaminergic polymorphic ventricular tachycardia experts?

Can catecholaminergic polymorphic ventricular tachycardia go into remission?

In medicine the word "remission" is often used to refer to a condition where there is no evidence of ongoing disease activity. The signs and symptoms of catecholaminergic polymorphic ventricular tachycardia (CPVT) are typically not constant, but serious concern is warranted for sudden CPVT events that may be triggered by activity or emotional stress. It has been estimated that 83% of people with CPVT will experience CPVT related symptoms. Symptoms of CPVT often present in childhood (75% by age 20),^[1]^[2] however CPVT has presented in older adults as well.^[2]

Last updated: 1/25/2013

Do all people with catecholaminergic polymorphic ventricular tachycardia require treatment?

It has been recommended that all people clinically diagnosed with catecholaminergic polymorphic ventricular tachycardia (CPVT) receive treatment. Some individuals who have never had or demonstrated symptoms of CPVT, for example asymptomatic family members with CASQ2 gene mutations, may still benefit from treatment.^[3] We recommend that you speak with your healthcare provider regarding your treatment options.

Last updated: 1/25/2013

Can people with catecholaminergic polymorphic ventricular tachycardia play sports?

It has been generally recommended that young people with genetic heart disease be "cautioned against virtually all forms of vigorous physical activity."^[1] We recommend that you speak with your healthcare provider for further advice regarding your activities and restrictions.

Last updated: 1/25/2013

How might catecholaminergic polymorphic ventricular tachycardia be treated?

Treatment of adults with catecholaminergic polymorphic ventricular tachycardia (CPVT) related arrhythmias typically involves the use of beta blockers. An implantable cardioverter-defibrillator (ICD) and beta blocker treatment may be considered for people with CPVT who have experienced a heart attack, fainting spells, or sustained abnormal heart rhythms on beta blockers alone. If these individuals still experience heart rhythm abnormalities flecainide and/or verapamil may also be recommended. People whose condition remains resistant to therapy may be counseled regarding further treatment options, such as left sympathetic denervation.^[3]

GeneReviews provides a current, expert-authored, peer-reviewed, full-text articles which includes information on treatment of CPVT at the following link:
http://www.ncbi.nlm.nih.gov/books/NBK1289/#cvt.Management

Last updated: 1/25/2013

Can catecholaminergic polymorphic ventricular tachycardia be cured?

Effective treatments are available for catecholaminergic polymorphic ventricular tachycardia, however there is currently no targeted treatment that can cure CPVT at this time.

Last updated: 1/25/2013

Are there any research studies involving catecholaminergic polymorphic ventricular tachycardia?

Yes. To learn more about research studies and clinical trials involving catecholaminergic polymorphic ventricular tachycardia, please visit the Clinical Trials & Research tab at the following link:
http://www.rarediseases.info.nih.gov/GARD/Condition/4421/Catecholamineinduced_polymorphic_ventricular_tachycardia.aspx

Last updated: 1/25/2013

How can I find catecholaminergic polymorphic ventricular tachycardia experts?

While we do not have a list of doctors who specialize in treating catecholaminergic polymorphic ventricular tachycardia, we have developed the following information page titled, How To Find An Expert that has tips for finding knowledgable professionals, and may be a helpful resource.

Last updated: 1/25/2013

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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References References

Pflaumer A, Davis AM. Guidelines for the diagnosis and management of catecholaminergic polymorphic ventricular tachycardia. Heart, Lung and Circulation. February 2012;21(2):96-100; http://www.ncbi.nlm.nih.gov/pubmed/22119737. Accessed 1/25/2013.
Napolitano C, Priori SG, Bloise R. Catecholaminergic polymorphic ventricular tachycardia. GeneReviews. October 14, 2004; http://www.ncbi.nlm.nih.gov/books/NBK1289/. Accessed 1/25/2013.
Buxton A. Catecholaminergic polymorphic ventricular tachycardia and other polymorphic ventricular tachycardias with a normal QT interval. In: Basow, DS (Ed). UpToDate. Waltham, MA: UpToDate; 2012;

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