Arrhythmogenic right ventricular dysplasia (ARVD) is a rare form of cardiomyopathy. It occurs when the heart muscle tissue in the right ventricle dies and is replaced by fat or scar tissue. As a result, the heart cannot pump properly. Patients with ARVD often have arrhythmias, which can increase the risk of sudden cardiac death. ARVD usually occurs in teens and young adults. It has also been linked to sudden cardiac death in young athletes.
What are the causes?
Doctors do not know what causes ARVD. It often runs in families, but it can occur in people with no family history of the condition. ARVD may also be caused by congenital conditions (those that are present at birth) and viral or inflammatory myocarditis.
What are the symptoms?
Symptoms of ARVD include palpitations and fainting after physical activity, ventricular arrhythmias, dizziness, and lightheadedness. Heart failure may also a symptom of ARVD.
How is it diagnosed?
- Holter monitoring gets a nonstop reading of your heart rate and rhythm over a 24-hour period (or longer). You wear a recording device (the Holter monitor), which is connected to small metal disks called electrodes that are placed on your chest. With certain types of monitors, you can push a "record" button to capture your heart's rhythm when you feel symptoms. Doctors can then look at a printout of the recording to find out what causes your arrhythmia.
- Echocardiography can be used to show the size of your heart and how much damage there is. Echocardiography is also used to see if there is decreased heart wall motion (called hypokinesia or hypokinesis).
- Computed tomography (CT) scanning gives slice-like pictures of your heart that can be used to show how the heart is working.
- Magnetic resonance imaging (MRI) can give detailed pictures of the heart and its various structures.
- Electrophysiology studies (EPS) are usually done in a cardiac catheterization laboratory. A long, thin tube called a catheter is inserted into an artery in your leg and guided to your heart. A map of electrical impulses from your heart is sent through the catheter. This map helps doctors find out what kind of arrhythmia you have and where it starts.
How is it treated?
There is no known cure for ARVD. Instead, treatment is focused on relieving the symptoms. You will likely be given antiarrhythmic medicines. If there is a risk for sudden death, your doctor may recommend an implantable cardioverter defibrillator. If you have ARVD, you will need to ask your doctor about exercising or participating in sports.
See also on this site: Cardiomyopathy
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National Heart, Lung and Blood Institute
Updated August 2012