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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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 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:
The most common symptom of ARVC is an irregular heartbeat.
Other symptoms include:
These changes in heart rate can lead to:
Some people suffer symptoms of heart failure with ARVC, including:
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:
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.
The goals of AVRC treatment are to:
At UPMC we have a number of ways to meet the treatment goals.
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.
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.
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.
After ARVC treatment, you'll need routine follow-up care with a heart specialist.
Last reviewed by a UPMC medical professional on 2024-10-01.
