Arrhythmogenic Right Ventricular Cardiomyopathy

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What is Arrhythmogenic Right Ventricular Cardiomyopathy?

Arrhythmogenic [uh-RITH-muh-JEN-ik] right ventricular [ven-TRIK-yoo-luhr] cardiomyopathy [kar-dee-oh-my-AH-puh-thee], called ARVC for short, is a kind of heart disease that is most common in adults. Pediatric ARVC is a very rare form of this disease that affects children under the age of 18. ARVC is also known by the following terms:

  • Arrhythmogenic right ventricular dysplasia [dis-PLEY-zhuh]
  • Right ventricular dysplasia
  • Right ventricular cardiomyopathy

The myocardium [my-oh-KAR-dee-uhm] is the muscular wall of your heart. It performs the squeezing that forms the heart beat and pumps blood through the body. If your child has ARVC, the muscle cells in the myocardium are replaced by fatty tissue. This can cause the right side of the heart to look thicker in certain places. As the disease gets worse, the heart wall gets thin, and the left part of the heart can also have muscle damage. When this happens, the heart can start to skip beats or become weaker (heart failure).


ARVC is a disease in the heart, which means that it can affect the heart itself, and also cause symptoms in other parts of the body. Some common symptoms include:

  • Heart flutter or pounding in the chest
  • Feeling lightheaded
  • Fainting
  • Shortness of breath
  • Swelling in the legs
  • Swelling in the abdomen (belly)

If your child has a less serious form of this disease, they might not have any symptoms at all, or they might only notice symptoms when they play sports or do other intense exercise. Even if there are no symptoms, there is still a risk of serious problems, including heart failure and sudden death.

ARVC is a progressive disease, which means that it gets worse over time. Children with ARVC might not have as many symptoms because they are young and the other parts of their heart and body are healthier. As they become adults and move into middle age, they might notice more symptoms. If your child has ARVC, their doctor, cardiologist, and other members of the medical team will talk to you about managing and keeping track of symptoms.

When to See a Doctor

ARVC is very rare in people under the age of 18. However, it has many symptoms in common with other heart problems that can also be serious. If your child is having any of these symptoms, schedule an appointment with their doctor right away.  In some cases, the doctor may refer your child to a cardiologist, a doctor who specializes in diagnosing and treating heart problems.


ARVC is a genetic condition, which means it is caused by changes in your child’s genetic code. This code is used by the body to create new cells, keep organs functioning, and control growth and development. Errors in this code, are common, but most don’t cause any problems. However, an error in the instructions for creating muscle cells can causes disorders like ARVC.

ARVC runs in families. This means that a mother or father can pass the mutation onto their child. If both the mother and father have the gene, the child will probably have ARVC.

Diagnosis and Tests

If your doctor thinks your child might have ARVC, they will give your child a physical exam and ask you and your child questions about the symptoms. Testing may be recommended to rule out more common conditions that can cause the same symptoms. Some of the questions your doctor might ask you or your child include:

  • What are your child’s symptoms?
  • Do the symptoms get worse when your child exercises or play sports?
  • When did the symptoms start? Are they getting worse over time?
  • Has a close relative died before the age of 40 from heart related problems?

After the initial exam, your doctor might order some tests to diagnose ARVC and other heart conditions. These can include:

  • Electrocardiogram [ih-LEK-trow-KAR-dee-oh-GRAM] or ECG
  • Signal average ECG
  • Exercise stress test
  • Echocardiogram [eh-koh-KAR-dee-oh-GRAM]
  • Cardiac MRI
  • 24-hour Holter monitor


Treatment for ARVC is focused on dealing with the parts of the disease that can cause heart failure, like abnormal heartbeat. If your child has ARVC, their doctor will probably refer them to a cardiologist, a doctor who specializes in heart problems. The cardiologist might order a number of treatments that can help with the symptoms and problems caused by this disease. Treatments can include:

  • Implantable cardioverter [KAR-dee-OH-ver-TUHR] defibrillator [dee-FIB-rill-AY-tohr] (ICD). An ICD is a small electronic device that keeps track of your child’s heartbeat and gives a small electric shock to the heart if it stops beating, or if its beating is not regular. The electricity helps the heart get back to a normal beating pattern.
  • Antiarrhythmic [an-tee-uh-RITH-mik] medicines. These medicines help keep your child’s heart beating at a normal rate. If your child is prescribed an antiarrhythmic, they will probably need to take it for the rest of their life.
  • Ablation [ahb-LAY-shun]. Your child’s doctor can do a scan of their heart to look for specific parts of the myocardium that are causing the irregular heartbeat or other symptoms. Cardiac ablation can be used to get rid of the heart tissue in a specific area that is causing problems.


ARVC itself can’t be prevented, but with early detection, your child’s doctor, cardiologist, and medical team will have more time to help slow down the progress of the disease and treat the serious symptoms that ARVC can cause, like arrhythmia or heart failure.
Arrhythmogenic [uh-RITH-muh-JEN-ik] right ventricular [ven-TRIK-yoo-luhr] cardiomyopathy [kar-dee-oh-my-AH-puh-thee], or ARVC, is a kind of heart disease that causes the muscles in the heart to weaken and be replaced with fatty tissue. This can result in an arrhythmia [uh-RITH-mee-uh] (irregular heartbeat), causing your child to get lightheaded or dizzy, especially when they exercise. ARVC in children is very rare but can cause heart failure and death.