Cardiomyopathy

Cardiomyopathy

What is cardiomyopathy?

Cardiomyopathy is a group of conditions that affects the muscle of the heart and reduces its ability to pump blood around the body. It can alter the shape of the heart or the size and thickness of the muscle walls.

Cardiomyopathy is often an inherited disease caused by a mutation in a person’s DNA that affects how the heart develops, but it can also be caused by viral infections, diseases that attack the immune system and some medical treatments such as those used to treat cancer.

There are six types of cardiomyopathy, as follows:

Dilated cardiomyopathy (DCM)
This occurs when the muscle wall of the left ventricle becomes enlarged and stretched, thus weakening the heart. DCM can affect anyone but if it develops during pregnancy it may be referred to as peripartum cardiomyopathy.

Hypertrophic cardiomyopathy (HCM)
This occurs when the muscle of the ventricle thickens, known as ‘hypertrophy’, which makes the ventricle smaller. As a result the heart has to work harder and HCM can also block the flow of blood out of the heart.

Arrhythmogenic right ventricular cardiomyopathy (ARVC)
Also known as arrhythmic cardiomyopathy, ARVC occurs when heart muscle cells are not joined together properly. As a result the cells die and get replaced by scar tissue or fat, which makes the heart muscle stretched, thinner and weaker.

Restrictive cardiomyopathy (RCM)
This occurs when the heart muscle becomes stiff and cannot relax properly, which makes it harder for the top chambers to fill with blood. The chambers then become enlarged and blood cannot flow properly.

Left ventricular noncompaction (LVNC)
LVNC is as a result of how the heart muscle develops in an unborn baby. The muscle cells do not pack together and this causes small indentations in the muscle, which appears spongy, and affects the performance of the heart.

Takotsubo or ‘broken heart’ syndrome
This usually happens during a time of extreme stress, hence the name ‘broken heart’, when the left ventricle becomes enlarged and weakened. It is often only temporary, and usually gets better with time.

What are the symptoms of cardiomyopathy?

Many people have cardiomyopathy without displaying any symptoms, whereas others may experience breathlessness, dizziness, fainting, tiredness, chest pain, palpitations, and in some cases swelling in the abdomen and ankles.

What assessment do I need if I have cardiomyopathy?

Your cardiologist will explore your family’s health history and you would normally have an ECG (electrocardiogram) and either an echocardiogram or MRI/CMR scan. As cardiomyopathy can be inherited, it may be that we would recommend that your first degree relatives are advised to have screening tests as well (ECG and echocardiography).

What are the treatments for cardiomyopathy?

Unfortunately there is no cure for cardiomyopathy; however many people take medication to alleviate the symptoms. Medication regularly prescribed includes anti-coagulants to thin the blood and reduce the risk of blood clots, diuretics to reduce the build-up of fluid, and beta-blockers to reduce the heart rate.

Some people will have a medical device inserted into their body during a surgical procedure, either a pacemaker that controls the rhythm of the heart or an ICD (implantable cardioverter defibrillator) which controls the rhythm and shocks the heart if the rhythm changes from normal to a dangerous rhythm.

In a small number of cases, people undergo surgery to remove some heart muscle if it affects blood flow, and in very few situations people have to have a heart transplant.