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Conditions

Catecholaminergic polymorphic ventricular tachycardia (CPVT) 

CPVT is a rare inherited condition that affects your heart’s rhythm. When someone with CPVT is under physical or emotional stress, it can trigger an abnormally fast heartbeat. CPVT often first appears in childhood and in young people.   

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CPVT causes

CPVT is known to be caused by a genetic change 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 abnormal heart rhythms  also known as arrhythmias. In particular, CPVT causes a very fast heart rhythm called ventricular tachycardia.

CPVT symptoms

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 with CPVT have no symptoms.

Speak to your doctor if your symptoms start to worsen.

If you're with someone when they collapse suddenly and are not breathing normally, call 999 immediately and 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. 

CPVT diagnosis

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 do not have symptoms of the condition.

CPVT treatment

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 help treat dangerous heart rhythms.
  • Take beta-blockers help to slow down your heart rate and help to prevent life-threatening arrhythmias. 

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

About 1 in 3 people need to be fitted with an ICD if medicine 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.

Genetic Information Service

If you have further questions about inherited heart conditions, call our  BHF Genetic Information Service on 0300 456 8383 to speak to one of our specialist nurses. Lines are open from 9am to 5pm Monday to Friday (charged at a rate similar to 01 or 02 calls).

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Being diagnosed, or living with a heart or circulatory condition can be overwhelming, but we're here to help.

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To find out more, or to support British Heart Foundation’s work, please visit www.bhf.org.uk. You can speak to one of our cardiac nurses by calling our helpline on 0808 802 1234 (freephone), Monday to Friday, 9am to 5pm. For general customer service enquiries, please call 0300 330 3322, Monday to Friday, 9am to 5pm.

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