Catecholaminergic polymorphic ventricular tachycardia (CPVT) 

Catecholaminergic polymorphic ventricular tachycardia (CPVT) explained

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited heart rhythm disturbance. It usually happens when someone is under physical or emotional stress and can trigger an abnormally fast heartbeat. CPVT often first appears in childhood and in young people.

What causes CPVT?

CPVT is known to be caused by a faulty gene passed down from one of your parents. Within your heart cells, proteins regulate the release of calcium ions. If these proteins function abnormally, it can lead to a rise in the level of calcium inside your cells.

The extra calcium can cause arrhythmias - also known as abnormal heart rhythms. In particular CPVT causes a very fast heart rhythm called ventricular tachycardia. 

Symptoms of CPVT

The most common symptoms of CPVT are:

• blackouts
• palpitations
• dizziness
• fainting.

These can be brought on by too much physical activity and stressful times, which can cause an increase in the amount of adrenaline running through your blood.

Some people have no symptoms of CPVT.

If you're with someone when they collapse suddenly and aren't breathing normally, call 999 immediately and try to perform cardio-pulmonary resuscitation (CPR) until an ambulance or medical help arrives. If you are untrained in CPR, the 999 operator will be able to talk you through the process.

• CPR is an essential life skill. Learn it with us from home, for free, in 15 minutes. 

How is CPVT diagnosed?

If you experience symptoms, your doctor will refer you to a specialist who will do an exercise test. This is an ECG that records your heart's electrical activity whilst you exercise, to try and induce the abnormal rhythm in a controlled, safe way.

Someone may also have an resting ECG and 24 hour monitor.

You'll need to discuss the level of activity that is safe for you with your doctor. It’s very important that families affected by CPVT receive an accurate assessment, diagnosis, treatment and support from specialists in a clinic for inherited heart conditions.

Genetic testing can also be done if someone in your family has already been diagnosed with CPVT even if you don’t have symptoms of the condition.

It’s important to speak to your doctor if your symptoms start to worsen.

What treatment is available?

For long-term management doctors may advise that you:

• avoid intense exercise and competitive sports
• take a daily medicine like beta-blockers or calcium channel blockers
• have an implantable cardioverter defibrillator (ICD) implanted to monitor your heart rate and stop dangerous heart rhythms. 

Beta-blockers help to slow down your heart rate and help to prevent life-threatening arrhythmias. 

If someone is having ventricular tachycardia (the very fast heart rate), they are usually given an electrical cardioversion/defibrillation (shock) to return the heart rhythm back to normal. Having a VT is an medical emergency so needs treatment right away. 

Roughly 1 in 3 people need to be fitted with an ICD if medication is not enough to reduce the risk of an dangerous abnormal heart rhythm.  If this is the case you will have to avoid contact sports and it could affect the type of work you do, especially if you work with magnetic or power tools.

If you have further questions about inherited heart conditions, our Genetic Information Service can help you. Lines are open from 9am to 5pm Monday to Friday (charged at a rate similar to 01 or 02 calls).

Genetic Information Service

Call our BHF Genetic Information Service on 0300 456 8383 to speak to one of our specialist nurses for information about inherited heart conditions and how they can affect you and your family.

Heart Helpline and other support:

• Call our Heart Helpline on 0808 802 1234 between Monday to Friday, 9am to 5pm.
• Speak to others with heart conditions by joining a support group or online community.
• Sign up to our Heart Matters magazine and online information packed with health and lifestyle advice.

Our scientists are helping to beat heartbreak

Our research funding has already helped scientists find a faulty gene that causes CPVT.

This research was only possible through generous donations from the public. Your donations help us fund hundreds of top scientists all over the UK, working on more than a thousand different research projects, but there is still lots of work to do in the effort to beat heartbreak forever.

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