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Arrhythmogenic Right Ventricular Cardiomyopathy

 Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited disease that causes the heart to beat irregularly. It's also known as arrhythmogenic right ventricular dysplasia (ARVD).

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What Is Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)? 

ARVC mostly affects the right lower chamber of the heart but can also affect the left one.

The heart muscle tissue becomes diseased, causing an increase in both fat and scar tissue in the lower right chamber.

This can cause abnormal heart rhythms and weaken the heart's lower right chamber.

ARVC causes

ARVC occurs in about 1 in 5,000 people.

Mutations or changes mainly in the genes that join the heart muscle's cells together (desmosomes) cause ARVC.

These gene changes:

  • Affect the proteins that secure the bonds between heart cells and keep the heart muscle strong.
  • Can disrupt the heart cell interactions and injure the heart cells, causing an abnormal heartbeat.
  • Stretch out the heart's lower right chamber, preventing the heart from pumping enough blood.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Symptoms and Diagnosis

The most common symptom of ARVC is an irregular heartbeat.

Other symptoms include:

  • Fast heartbeat.
  • Heart palpitations.
  • Fluttering feeling in the heart.

These changes in heart rate can lead to:

  • Fainting
  • Dizziness
  • Nausea
  • Lightheadedness

Some people suffer symptoms of heart failure with ARVC, including:

  • Swelling in the legs or feet.
  • Shortness of breath.
  • Fatigue.

Diagnosing ARVC

Your doctor can diagnose ARVC with a series of tests and exams.

First, they'll do a physical exam and take a detailed family history.

Your doctor will also order tests to check your heart function, such as:

  • EKG.
  • 24-hour Holter monitor.
  • Echocardiogram.
  • Exercise stress test.
  • MRI.
  • CT scan.
  • Electrophysiology study.

Because ARVC is an inherited disorder, your doctor may also suggest genetic testing.

Before the test, you'll talk to a genetic counselor who will explain the risks and benefits of testing. They'll also tell you about any limits in interpreting the results.

If you carry a genetic mutation for ARVC, there's a risk that you may pass the gene along to your children. Doctors may suggest that your parents, siblings, and children also have testing.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Treatment

The goals of AVRC treatment are to:

  • Control abnormal heart rhythms.
  • Prevent or reduce heart failure.
  • Avoid sudden cardiac arrest.

At the UPMC Center for Inherited Heart Disease, we have a number of ways to meet the treatment goals.

Medicine to treat ARVC

Your doctor may prescribe drugs to help keep a normal heart rhythm. These include beta blockers or antiarrhythmic agents.

ACE inhibitors treat signs of heart failure and can also slow its progression.

Implanted cardioverter defibrillator (ICD)

An implanted ICD the most common treatment for ARVC for people at risk of sudden death.

The device tracks the heart's rhythm. If that rhythm becomes irregular, the ICD sends an electric shock to return the heart to a normal rhythm.

You'll have to see your doctor regularly for imaging and to make sure treatment is working.

Catheter ablation

In this treatment, a doctor inserts a thin tube (catheter) into a vein in the leg and threads it to the heart.

The tube has tips that get heated and destroy the tissue causing your irregular heartbeat.

Living with ARVC

After ARVC treatment, you'll need routine follow-up care with a heart specialist.

Why Choose UPMC for Heart Care?

The Center for Inherited Heart Disease at UPMC:

  • Takes a complete, family-based approach to caring for people with inherited heart disease.
  • Provides a team of heart doctors, children's heart specialists, genetic counselors, surgeons, and others.
  • Helps you get answers, whether you have symptoms or suspect ARVC from a genetic testing.