Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) | MedStar Health

A rare genetic abnormality of the heart muscle disease

Also known as ARVC, this disease damages cells in the right chamber of the heart, which are replaced over time with fat and scar tissue. This leads to abnormal heart rhythms, or arrhythmias. The disease is also known as arrhythmogenic right ventricular dysplasia (ARVC or ARVD/C).

ARVC is a leading cause of sudden death in high school and college-aged athletes, though these incidents are rare. Most people with ARVC are diagnosed in their 20s or early 30s, but it can affect anyone of any age. Estimates of how many people have ARVC range from one in 1,000 to one in 5,000 people.

Symptoms

Most people with ARVC don’t experience noticeable symptoms. Sadly, the first symptom may be sudden cardiac death.

If mild symptoms are noticed, they might resemble symptoms of other arrhythmias or heart failure. Symptoms may include:

  • Chest pain, or angina

  • Dizziness
  • Extreme fatigue
  • Lightheadedness or fainting (syncope)

  • Nausea
  • Palpitations, or the sensation that the heart rate is skipping, slowing down, or racing

  • Shortness of breath at rest and during exercise
  • Swelling in the legs, feet, or stomach area

Causes

ARVC is a genetic condition, which means a specific gene mutation is passed down between family members. Research suggests that infections of the heart muscle also can cause ARVC.

The physicians in our Electrophysiology Program care for patients with rare and complex arrhythmias such as ARVC. With careful monitoring and sticking to a personalized care plan, most people who have ARVC don’t die from it.

If you are diagnosed, your first-degree relatives, such as your children, parents, and siblings, may want to consider genetic testing.  This is a personal decision, so talk it over with your doctor before making that choice.

We recommend that people who have ARVC take certain precautions to prevent their condition from worsening, including:

  • Avoiding strenuous activity
  • Avoiding stimulants including caffeine, nicotine, and certain decongestants

Tests

Angiogram (Angiography)
An angiogram is a special X-ray taken as a special dye is injected through a thin, flexible tube called a catheter to detect blockages or aneurysms in blood vessels.
Cardiac Catheterization

Cardiac catheterization is a minimally invasive way to diagnose and treat a variety of heart and vascular conditions by guiding thin, flexible tubes called catheters through blood vessels to problem areas.

Carotid Duplex Ultrasound

Carotid duplex ultrasound uses Doppler and traditional ultrasound to assess blood flow in the arteries that supply blood to your brain.

Chest X-ray

Chest X-rays use a small dose of radiation to create pictures of the structures inside the chest, including the lungs, heart, and chest wall.

Computerized Tomography (CT) Scan

The cardiac computed tomography scan, or cardiac CT, uses X-rays to create three-dimensional images of your heart and blood vessels.

Echocardiogram

An echocardiogram uses high-frequency sound waves to create images of your heart.

Electrocardiogram (ECG)

An electrocardiogram, also known as an ECG, measures the heart’s electrical activity.

Electrophysiology Testing

Electrophysiology testing is used to evaluate the cause and location of an abnormal heartbeat (known as an arrhythmia).

Event Monitors

An event monitor is a small device that records the heart’s electrical activity. It’s similar to an electrocardiogram, but where an electrocardiogram takes place over a few minutes, an event monitor measures heart rhythms over a much longer time.

Heart Biopsy

In a heart biopsy, your doctor will remove small samples of your heart muscle tissue to monitor heart function or diagnose a problem.

Holter Monitors

A Holter monitor is a small device that records the heart’s electrical activity. It’s similar to an electrocardiogram, but whereas an electrocardiogram records over a few minutes, a Holter monitor records over the course of a day or two.

Loop Recorder

A loop recorder is a device that’s implanted underneath the skin of your chest to record your heart rhythm for up to three years. 

Magnetic Resonance Imaging (MRI)

Magnetic resonance imaging, better known as cardiac MRI, is a combination of radio waves, magnets, and computer technology to create images of your heart and blood vessels.

Stress Tests

Stress tests are used to assess how your heart works during physical activity. There are several types of stress tests, including treadmill or bike stress tests, nuclear stress tests, stress echocardiograms, and chemically induced stress tests.

Transesophageal Echocardiogram (TEE)

Transesophageal echocardiogram allows us to take very detailed images of your heart structure from a probe in your esophagus.

Treatments

Though there is no cure for this disease, our heart and vascular teams can create individualized plans to manage symptoms of the condition. Your treatment plan may include:

Implantable Cardioverter Defibrillator (ICD)

An implantable cardioverter defibrillator (ICD) is a device implanted below your collarbone that monitors your heart’s rhythm. When it detects an abnormal rhythm, it delivers an electrical impulse or shock to the heart to correct it.

Electrical Cardioversion

Electrical cardioversion uses electrical shocks to treat heart rhythm disorders.

Left Ventricular Assist Device (LVAD)

A left ventricular assist device (LVAD) helps the heart pump blood more effectively during end-stage heart failure.

Additional information

Genetic Counseling and Testing for Heart Conditions

Our cardiogenetic experts work to improve early detection and treatment for patients at risk for certain genetic heart conditions.

Read our Cardiovascular Performance & Outcomes Booklet

Ask MHVI

Have questions for our heart and vascular program? Email us at AskMHVI@medstar.net.