Arrhythmogenic cardiomyopathy (ACM) is a heart condition that affects the muscle in the heart. The heart is made up of four chambers. The bottom two chambers are called the ventricles. The ventricles are the pumping chambers of the heart which send blood out from the heart and around the body. ACM can affect the right or left ventricle of the heart, or both.
ACM is an inherited condition, meaning it is caused by a genetic change present at birth that can be passed down through a family.
Past names of ACM
ACM is sometimes also called arrhythmogenic right ventricular cardiomyopathy (ARVC) or dysplasia (ARVD). We now recognise that the left side of the heart is also commonly affected.
In a person with ACM, the normal heart muscle is replaced by scar tissue, and fat. These changes to the heart muscle can cause two main problems:
ACM can be diagnosed by a cardiologist (heart doctor), and is usually diagnosed through testing that checks how well your heart is working. Your cardiologist may also repeat these tests as part of your ongoing management.
Testing may include, but is not limited to:
These changes in the heart can lead to a range of symptoms that will differ from person to person. Even people in the same family may experience different symptoms of ACM.
Some people with ACM may experience no symptoms while others may experience a range of symptoms.
Symptoms can include:
In serious cases, ACM can lead to heart failure, or sudden cardiac death. There are often no obvious symptoms prior to a sudden cardiac death.
Our bodies are made up of millions of cells. Each cell contains a copy of our genetic information. Our genetic information is the instructions for our body deciding how we will grow and function. Each instruction is called a gene. We have two copies of all our genes.
We share our genetic information with our families as we receive half our genetic information from each of our parents.
In ACM, one copy of a gene that is involved in your heart function may contain a genetic change (variant) that stops the heart from working as it should. The genetic variant changes the instructions sent out to the body and may cause your cardiomyopathy.
ACM is best managed by a team of healthcare professionals. This may include your GP, a cardiologist, a psychologist, and a cardiac genetic counsellor. Each member of your healthcare team plays a different and important role in caring for you, and your family. Management of your cardiomyopathy is very personal, your management plan will likely be different from other members of your family with the same diagnosis.
