Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare heart condition that can run in families. It’s a problem with the heart’s electrical system that can cause the heart to suddenly beat abnormally fast. CPVT is linked to sudden cardiac death in otherwise healthy people.

CPVT can present during infancy, childhood or adolescence and we try to diagnose it as early as possible. With the right treatment, we can prevent life-threatening episodes.

The heart specialists here at Lurie Children’s are doing extensive research into the genetics behind CPVT. Though it’s a rare condition (1 in 10,000), we have a lot of experience treating CPVT.

What are CPVT Symptoms?

With CPVT, the heart has a normal structure and a normal rhythm when at rest. But when a person’s nervous system is suddenly stimulated, their heart rhythm can become dangerously irregular. This can happen in situations that cause a surge of adrenaline, such as if a person is startled or is exercising.

Fainting, called syncope, is the main symptom. (There are far more common reasons for fainting, though.) Sometimes the first symptom is sudden cardiac arrest.

How We Diagnose & Treat CPVT

To diagnose CPVT, we need to record the heart’s rhythm when it’s being put under stress. We may use medication to do this, or ask a child to run on a treadmill. This is called an exercise stress test.

Finding the arrhythmia is a key piece of identifying CPVT. Since CPVT runs in families, we also use genetic testing to diagnose CPVT. We even have a Cardiovascular Genetics program that evaluates and treats families with genetic heart disease.

CPVT usually involves one or more of these treatments:

  • Medications: There are many available medications for CPVT. We have the expertise to help determine which is the best for a child.
  • Exercise restriction: Exercise is one of the triggers for the dangerous heart rhythm. We work with families to find a safe exercise level that meets the family’s goals.
  • Surgery: For some children, we may recommend a surgery that prevents the heart from seeing those surges of adrenaline. We sometimes implant a device that can shock the heart back to a normal rhythm. This isn’t the right treatment for everyone, though.

Why Choose Lurie Children’s for Treating CPVT?

Each year, we help families navigate CPVT. When it comes to managing this condition, Lurie Children’s stands out in several ways:

  • Expertise with diagnosis: CPVT can be complex to diagnose. Our cardiac teams work together to get answers for families, so that we can treat immediately.
  • Research into sudden cardiac death: Through National Institute of Health (NIH) studies, we’ve been at the forefront of learning about new types of heart rhythm-related genetic diseases. We’re learning more about genetic causes every day, which impacts how we treat conditions like CPVT.
  • Surgical excellence: For surgeries and other pediatric heart procedures, our outcomes are among the best in the nation.
  • Top ranked heart care: We’re a top-ranked pediatric heart center by U.S.News & World Report. We treat the range of pediatric heart conditions, including the most complex.

Long-Term Effects of CPVT

One of the most difficult aspects of CPVT is identifying it before a child has an episode. That’s why we are so focused on learning genetic causes.

Once we diagnose CPVT, we can treat right away. We know that aspects like exercise restriction can be challenging. Not being able to participate in certain activities can affect someone’s quality of life. This is why we use a shared decision-making model to devise a plan to help a child live their best life.

For most people with CPVT, we can find a strategy that results in no more life-threatening episodes.

Heart Center Family Resource Guide

To help prepare families for their care with Lurie Children's Heart Center, we have compiled a list of resources about treatment and recovery. Learn how to get ready for an inpatient stay or outpatient visit, and read about our support services for patients and families.

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