Arrhythmogenic right ventricular cardiomyopathy                

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 become thin and stretched and so the heart doesn’t pump blood around the body properly.

ARVC can also cause abnormal heart rhythms as the electrical impulses are slowed down as they pass through the muscle wall unevenly. It usually takes many years for this to happen so most people are not diagnosed until later in life and the condition is usually progressive – which means that it will get worse over time.

1. Concealed phase

Here there are generally no symptoms - but the person may be at risk of sudden death, particularly during extreme exertion.

2. Overt phase

Symptoms include:

• Palpitations
• Light-headedness
• Fainting
• Risk of sudden death on exertion
• Abnormal heart rhythms

3. Weakening of the right ventricle

Symptoms include:

• Swollen ankles or legs
• Swelling in the abdomen
• Abnormal heart rhythms

4. Weakening of the left ventricle

Symptoms include:

• Breathlessness
• Fluid building up in the lungs, ankles, abdomen and other organs of the body - known as heart failure

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 cardiomyopathy are an ECG, which records the electrical activity of your heart, and an echocardiogram which show the pumping action of your heart. 

Your doctor may also arrange for you to have an exercise test, signal averaged electrocardiogram or MRI scan.

• 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

Most of the symptoms caused by the condition can be controlled by 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 is different for everyone.

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 an ordinary car and continue to work for example. You will not be able to drive an HGV or commercial passenger vehicle however and there may be some restrictions on your employment depending on your type of work. For more information and advice about living with arrhythmogenic right ventricular cardiomyopathy, download or order our booklet.