Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare condition that affects the heart muscle. It runs in families, so children may have it — though we rarely see ARVC in infants. We usually diagnose ARVC after the age of 20.

ARVC can cause abnormal heart rhythms and it is linked to sudden death. It’s important to find and treat this condition as early as possible. ARVC is sometimes just called AVC (and was previously called arrhythmogenic right ventricular dysplasia, or ARVD).

At Lurie Children’s, we’re experts at genetic testing for ARVC and we use the most up-to-date treatments for both children and adults.

What are Symptoms of ARVC?

ARVC interferes with how the individual cells of the heart muscle connect to each other. The cells don’t “stick” together as they should. Tissue that doesn’t belong in the heart works its way into the places where cells should be.

The heart starts to replace muscle with fat. The combination of abnormal muscle and fat where muscle should be enlarges the heart. These same changes can cause irregular heart rhythms, called arrhythmias.

People often seek a diagnosis because they learn of a family history of this condition. Early on, they may not have any symptoms. But ARVC can get progressively worse, causing symptoms like fluttering in the chest and fainting (called syncope).

How We Diagnose & Treat ARVC

Other conditions or types of cardiomyopathy can look like ARVC. This is why ARVC can be a complex diagnosis to make.

Lurie Children’s has expertise in both assessing genetic risk for ARVC and using pediatric heart imaging to confirm the diagnosis. We even have a Cardiovascular Genetics program that evaluates and treats families with genetic heart disease.

We use different medications to manage the various aspects of ARVC. Some medications aim to stop the heart from enlarging. Others work to better regulate the heart rhythm. 

Some older children and young adults may need intervention. This may mean implanting a device to keep their heart beating regularly. We may also recommend a procedure called cardiac ablation to treat abnormal heart rhythms.

Why Choose Lurie Children’s for ARVC Treatment?

Because ARVC is associated with sudden death, it’s important to catch it early and understand the consequences of the disease. Lurie Children’s has the exceptional ability to do this because of our:

  • Genetic expertise: Diagnosing diseases via genetic screening is complicated. We excel in this area, including a strong partnership with Northwestern University. Since ARVC is a disease that affects families, affiliation with an adult center matters.
  • Depth of experience diagnosing and treating ARVC: Though ARVC is rare, we have a great deal of experience with it. We’re especially good at navigating through the rapidly changing understanding of the disease, and stay current on all new findings and international guidelines.
  • Ongoing research: We participate in nationally funded research efforts related to cardiac imaging and genetics. Plus, we run a top program for device management in children with rhythm disorders. Lurie Children’s is also on the forefront of studying sudden cardiac death to learn how to prevent it.
  • Leading heart care: We’re a top-ranked pediatric heart center by U.S.News & World Report. We treat Chicago’s children, as well as kids from around the country and the world.

Long-Term Effects of ARVC

Without the right treatment, ARVC can lead to heart failure, or tragically, to sudden death. We work to prevent these things from happening. In fact, our top aim is to stop ARVC from progressing and causing heart damage.

In some cases, children with ARVC or children who have genetic markers of ARVC need to change their exercise plan. 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.

Every child is different, but we work as a team to find the right approach. We want to help young people with ARVC live as normal a life as possible.

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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