Catecholaminergic Polymorphic Ventricular Tachycardia

What is catecholaminergic polymorphic ventricular tachycardia?

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a heart rhythm disorder. In CPVT, your heart (the lower chambers known as the ventricles) beats too quickly when you exercise or feel extremely stressed. When your heartbeat speeds up, it’s harder for your heart to pump blood through your body as it should.

CPVT symptoms usually start when you’re a child. Without treatment, CPVT can lead to health complications, including cardiac arrest, when your heart suddenly stops beating. Cardiac arrest can be life-threatening. But with treatment, many people with CPVT live a high quality of life.

What is ventricular tachycardia?

Ventricular tachycardia is an abnormally fast heartbeat in your heart’s lower chambers (ventricles). When you have CPVT, you may experience ventricular tachycardia at certain times, such as after exercise.

How common is catecholaminergic polymorphic ventricular tachycardia?

CPVT is rare. Experts estimate about 1 in 10,000 people have it.

What causes catecholaminergic polymorphic ventricular tachycardia?

CPVT occurs because of a change (mutation) in one of your genes. Usually, you inherit this gene change from one or both parents. Sometimes, you don’t get the gene change from your parents, and it happens randomly.

What are the symptoms of catecholaminergic polymorphic ventricular tachycardia?

CPVT symptoms usually start when you’re a child. You may experience symptoms after intense exercise or when you feel strong emotions, such as extreme stress or excitement.

You may also:

• Faint or feel like fainting more often than is typical.
• Have fast and hard heartbeats (heart palpitations).
• Have seizures.

How is catecholaminergic polymorphic ventricular tachycardia diagnosed?

To diagnose CPVT, your healthcare provider uses tests that check your heart health, including:

• Echocardiogram (echo test) uses sound waves to check your heart’s structures and blood flow.
• Electrocardiogram (EKG) measures your heart’s electrical signals.
• Exercise stress test measures how your heart rate changes when you exercise.
• Imaging tests, such as MRI, cardiac catheterization or coronary angiography, get a better picture of your heart’s function and blood flow.
• Genetic tests look for the gene changes that lead to CPVT.

Your healthcare provider may also give you an ambulatory monitor to track your heart’s electrical activity. You wear the monitor for a few days or weeks as you go about your usual activities. The monitor can give your healthcare provider a more comprehensive picture of your heartbeat throughout the day.

How is catecholaminergic polymorphic ventricular tachycardia treated?

If your heart is in ventricular tachycardia, you may need an electrical shock (cardioversion) to restore typical rhythm. Electrical cardioversion in this case is usually an emergency treatment.

Long-term CPVT treatment focuses on keeping your heart in a regular rhythm. Your treatment plan may include:

• Medicines, such as calcium channel blockers (Cardizem®, Procardia®) or beta-blockers (Lopressor®, Corgard®) to prevent ventricular tachycardia.
• Implantable cardioverter defibrillator (ICD), a small device placed beneath your skin that uses electrical signals to keep your heart in rhythm.

You may also need to make some lifestyle changes, such as limiting strenuous exercise like running. Children with CPVT may need to avoid intense competitive sports.

How can I reduce my risk of CPVT?

CPVT occurs because of a gene mutation, so there’s no way to prevent it. But with management, you can live a high quality of life with CPVT.

If someone in your family has CPVT, consider genetic testing and counseling. A genetic counselor can help you understand what it means to have the condition and whether you could pass it to your children.

How can I prevent CPVT complications?

To lower your risk of CPVT complications, you can:

• Create a safe exercise plan with your healthcare provider.
• Limit caffeine, which can speed up your heart rate.
• Monitor your symptoms. Call your healthcare provider right away if you experience heart palpitations.
• Visit your healthcare provider regularly for ongoing care.

What can I expect if I have CPVT?

Without treatment, CPVT can lead to health complications. Some of these complications, such as sudden cardiac arrest, can affect life expectancy. Treatment can help keep your heart in typical rhythm and lower your risk of these complications.

If you have CPVT, you’ll need lifelong follow-up care to ensure your heart stays in regular rhythm. Contact your healthcare provider immediately if you experience CPVT symptoms, such as fainting after exercise.

What else should I ask my provider?

If you, or your child, are experiencing symptoms or received a CPVT diagnosis, you may want to ask your healthcare provider:

• What’s the most likely cause of these symptoms?
• What tests will help diagnose CPVT?
• What are the treatment options for CPVT?
• What at-home treatments help CPVT symptoms between appointments?

A note from Cleveland Clinic

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare heart rhythm disorder. It causes a too-fast heartbeat in your heart ventricles. Usually, you start noticing symptoms of CPVT in childhood. Children may faint, have seizures or feel like their heart is racing. These symptoms usually occur after intense exercise or emotional distress. Treatment can help people with CPVT stay in a regular heart rhythm and lead fulfilling lives.