Understanding Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) (Child)

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare genetic condition. It causes a very serious irregular heartbeat.


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What happens during CPVT?

A heartbeat usually starts in the upper right chamber of the heart (right atrium). In people with CPVT, a sudden, fast heart beat starts in the lower chambers of the heart (ventricles). This is called ventricular tachycardia (VT). With CPVT, the heart beats so quickly that it does not have enough time to fill between beats. As a result, less blood is pumped to the body and brain. This causes fainting (syncope) and other symptoms. If untreated, death may occur.

What causes CPVT?

CPVT can be passed down through families, especially if there is a family history of syncope or sudden death. It is from an abnormal gene. You can inherit it in different ways. One parent may pass down the gene. Or both parents may pass down the gene. If you have CPVT, talk with your family members about having genetic testing and heart testing. These tests can show undiagnosed CPVT in other family members.

What are the symptoms of CPVT?

CPVT usually occurs at a young age. Your child may have fainting (syncope), or near fainting. This happens most often during exercise, excitement, or when feeling strong emotions. Your child may also have symptoms such as:

  • Feeling lightheaded or dizzy

  • Feeling his or her heart beat fast or hard (palpitations)

  • Fainting

The episode of ventricular tachycardia may go away on its own. Or it may turn into ventricular fibrillation. This is a life threatening heartbeat that can potentially cause the heart to stop (cardiac arrest) and death. The person loses consciousness and stops breathing. In some cases, an episode of CPVT with cardiac arrest may be the first time the condition is found.

How is CPVT diagnosed?

CPVT is often diagnosed at a young age. This usually happens when a child has fainting with physical or emotional stress. The health care provider will ask about your child’s health history and symptoms. He or she will also ask about your family health history. Your child will have a physical exam. Your child may need to see a heart doctor (cardiologist). He or she may advise tests such as:

  • Electrocardiography (ECG). This is to check your child’s heartbeat and rhythm. ECG results are usually normal in people with CPVT.

  • Exercise stress testing. This is to check how the heart functions when stressed, such as during exercise.

  • Drug challenge. A medicine is given that makes the heart work harder, simulating exercising. This may be done if your child can’t exercise.

  • Holter monitoring. Your child wears a portable ECG monitor for one or more days.

  • Echocardiography. This test is done to check the heart to see if it has physical defects. It is an ultrasound of the heart to look at the structure and function of the heart muscle and its valves.

  • Genetic testing. This is to check for abnormal genes.