Arrhythmogenic right ventricular cardiomyopathy

A female nurse sits at bedside with woman as they look at information together.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare disease of the heart muscle.

ARVC is an inherited condition, which means that it's passed on through families. It is caused by a change or mutation in one or more genes. The chances of inheriting ARVC vary, and you might inherit the mutation but not develop the condition.

How does it affect your heart?

The cells of the heart muscle are held together by proteins. In people with ARVC, these proteins do not develop properly and so cannot keep the heart muscle cells together. The muscle cells become detached and fatty deposits build up in an attempt to repair the damage.
 
ARVC usually affects the right side of your heart but it can affect both sides. The changes to the heart muscle mean the walls of the ventricle - one of four chambers in your heart - become thin and stretched. This means your heart doesn’t pump blood around your body properly.
 
ARVC can also cause abnormal heart rhythms, because your heart’s normal electrical impulses are disrupted as they pass through areas of damaged and scarred muscle cells. It usually takes many years for this to happen so most people are not diagnosed until later in life.
 
The condition is usually progressive, which means that it will get worse over time.

What are the symptoms?

ARVC has four phases. Not everyone will develop all of the phases or be affected in the same way. Many people with the condition never develop any serious problems.

1. Concealed phase

There are small changes in the right ventricle.

2. Overt phase

There are noticeable structural changes to your heart muscle. These changes affect the pumping action of the heart and cause abnormal heart rhythms.

3. Weakening of the right ventricle

Your right ventricle becomes stretched and its pumping action becomes weaker. 

4. Weakening of the left ventricle

Your left ventricle is also affected. The pumping action of both your ventricles becomes weaker.
 
If you have ARVC, you may have one or more of the following symptoms:

How is ARVC diagnosed?

It can be difficult to diagnose ARVC, because the changes to the heart muscle are often very minor or build up in a 'patchy' pattern. Many of the changes and symptoms could also be caused by a number of other conditions, making diagnosis even more difficult.
 
Your doctor may suspect that you have ARVC because of your symptoms, or because of the results of a chest x-ray. Or you may be going for tests because someone else in your family has been diagnosed with the condition.
 
The most common tests for this cardiomyopathy are an ECG, which records the electrical activity of your heart, and an echocardiogram, which shows the pumping action of your heart.
 
Your doctor may also arrange for you to have an exercise test, signal averaged electrocardiogram or MRI scan.

Read about how we're funding research into the link between ARVC and a type of skin disease

How is ARVC treated?

There are three aims for treatment:

  • To identify those at risk of sudden cardiac death
  • To prevent or control arrhythmias (abnormal heart rhythms)
  • To improve the pumping action of the heart muscle

ARVC is not curable, but most of the symptoms caused by the condition can be controlled using medicines. You may also undergo treatments such as cardioversion or catheter ablation. Your treatment may stay the same for many years, or may change more frequently. It's different for everyone.

How will ARVC affect me?

Research has shown that with proper treatment and follow-up, most people with ARVC are able to control their symptoms and live a normal life.

Read about Carl, who hasn’t let ARVC stop him living life on the edge

Most people with ARVC are at low risk of sudden cardiac death. However, there are certain times – known as ‘hot phases’ – when the disease becomes more active and this increases the risk of sudden cardiac death. Unexplained dizzy spells, sustained palpitations, or blackouts can be signs of a hot phase.
 
If you experience any of these symptoms, you should see your doctor immediately. Hot phases may come and go, but they could be a sign that the disease is progressing. This is why regular monitoring and follow-up with your doctor are important. If you are at high risk of sudden cardiac death, your doctor may advise that you have an ICD fitted.
 
You may have to make some changes to manage your symptoms, but you should be able to drive a car and continue to work for example. However, you will not be able to drive an HGV or commercial passenger vehicle and there may be some restrictions on your employment depending on your type of work.

Want to know more?

Arrhythmogenic right ventricular cardiomyopathy

Dilated cardiomyopathy

Hypertrophic cardiomyopathy

     

Contact us

Call our BHF Genetic Information Service on 0300 456 8383 to speak to one of our specialist nurses for information about inherited heart conditions and how they can affect you and your family.

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