Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

What is arrhythmogenic right ventricular cardiomyopathy (ARVC)?

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an uncommon heart problem. It is most common in people younger than 35 years of age, but it can happen at any age. It is the leading cause of sudden death among young athletes.

The heart has 4 sections called chambers. The right ventricle is one of the lower chambers. If you have ARVC, a genetic defect causes muscle cells in your ventricle wall to die. They are replaced by fat and scar tissue. This can cause abnormal heart rhythms (arrhythmias).

Symptoms of ARVC

If you have ARVC, you may notice that your heart often beats very fast. You may feel like your heart is “skipping a beat” or that you can’t catch your breath. ARVC sometimes causes chest pain that may seem like a heart attack.

Other symptoms of ARVC include:

  • dizziness
  • fainting
  • shortness of breathwith activity
  • swelling of the legs, ankles, and feet.

You should see your doctor right away if you have any of these problems. Your doctor can test you for ARVC and other heart problems.

What causes ARVC?

ARVC is a genetic condition. It is caused by mutations in some of your genes. It can also be passed down in families. If you have a close relative who died before the age of 40 from heart-related problems, you may be at risk for ARVC. This could be a brother, sister, or parent.

How is ARVC diagnosed?

There is no single test for ARVC. Your doctor will do a physical exam. He or she will probably order a number of cardiac (heart) tests. These could include:

  • electrocardiogram (ECG)
  • signal averaged ECG (SAECG)
  • exercise stress test
  • echocardiogram
  • cardiac MRI
  • 24-hour Holter monitor.

Your doctor can use the results of these tests, your exam, and your family history to diagnose ARVC.

Can ARVC be prevented or avoided?

ARVC is a genetic, hereditary condition. It cannot be prevented or avoided.

ARVC treatment

There is currently no cure for ARVC. Treatment involves controlling abnormal heartbeats and managing signs of heart failure. Your doctor may give you medicine (called an antiarrhythmic) to keep your heart beating at a normal rate.

You might need an implantable cardioverter defibrillator (ICD). This small device monitors your heart’s rhythm and can give an electric shock to the heart if necessary. This will return it to a normal beating pattern.

Sometimes your doctor can do a study that determines which area of the heart is causing the abnormal rhythm. He or she can then eliminate (ablate) these areas. But ARVC is progressive, which means it continues to get worse as you get older. So this procedure does not permanently cure the condition.

A person with severe ARVC could need a heart transplant. But this is rarely necessary.

Living with ARVC

ARVC needs to be monitored and controlled closely by your doctor. Many people diagnosed with ARVC get an ICD. This will require you to have regular check-ups. You will also need yearly cardiac testing. It is a progressive disease, so it will continue to get worse as you get older. But when it is controlled with medicine and an ICD, you can live a fairly normal life.

Questions to ask your doctor

  • Do any of my family members need to be tested for ARVC?
  • What is the best treatment option for me? Will I need medicine? Surgery? A pacemaker?
  • How does ARVC affect my life expectancy?
  • Does ARVC put me at risk for other health problems?
  • Do I need to make any lifestyle changes?
  • Is it safe for me to exercise?

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