CARDIOMYOPATHY

Cardiomyopathy is a term indicating a problem with the heart muscle. It has to do with several conditions that affect the heart muscles and inhibit its ability to pump blood effectively around the body.

What Is Cardiomyopathy?

This is a cardiac issue that happens when several conditions affects the heart muscles in such a way that it impedes its blood pumping capability around the body. It can change the shape of the heart or the thickness and size of the muscle walls.

In some cases, this heart condition is hereditary as it is caused by an alteration in an individual's DNA that affects the development of the heart. However, other causes of this heart malfunction includes diseases that fight the immune system, viral infections, and some medical treatments like those used in the treatment of cancer.

There are a number of different types of cardiomyopathy including:

• Dilated cardiomyopathy (DCM) – Here the chambers of the heart grow bigger and the walls of the heart become stretched, causing their function reduce, making the heart weak. Sometimes DCM can be inherited but other times it occurs following a viral infection of the heart (myocarditis) or due to alcohol or other causes. The symptoms are those of heart failure, with breathlessness, difficulty lying flat, tiredness and swelling of the ankles.  Treatment is usually with drugs (ACE inhibitors, beta blockers, diuretics) but sometimes pacemakers and/or defibrillators may be useful.
• Hypertrophic cardiomyopathy (HCM) – In this condition the walls of the heart become thickened (known as hypertrophy). This is a genetic condition but sometimes it can arise from a new problem with a gene (i.e. there isn’t a history of the condition in the family).  The symptoms are often chest pain during exercise, palpitations, dizziness/collapses, and sadly sometimes sudden death.  This means it is important that patients with HCM are fully investigated to decide what treatment is required.  Some patients will need a defibrillator to protect them against dangerous heart rhythms.
• Arrhythmogenic right ventricular cardiomyopathy (ARVC) – Here there is infiltration of the heart muscle with fat and scar tissue, usually in the right side of the heart, but often also affecting the left side of the heart. This condition is usually genetic and the symptoms include palpitations, dizziness and unfortunately sudden death. A defibrillator is often required. 
• Restrictive cardiomyopathy (RCM) – In this condition the heart muscle stiffens and has difficulty in relaxing. The atria (chambers at the top of the heart) become enlarged. This can be genetic but can also arise from inflammatory conditions (such as sarcoidosis), amyloidosis, or previous chemotherapy and radiotherapy.  Symptoms are usually of breathlessness, tiredness and swelling of the ankles.  Treatment will include diuretics (tablets to make the kidneys remove more fluid). 
• Left ventricular noncompaction (LVNC) – This is a condition where the tissue making up the walls of the heart is not joined together normally, leading to it being “spongy” and having lots of channels and crevices. Although it can occur on its own, it is often found as part of other cardiomyopathies, particularly DCM.  It requires careful diagnosis as other conditions that cause the chambers of the heart to enlarge can be mistaken for LVNC.  Some patients with LVNC may require anticoagulation (blood thinners) to reduce the risk of stroke arising from clot building up in the spongy heart muscle.
• Takotsubo or 'broken heart' syndrome – Here there is a temporary problem with the middle and tip of the heart where they stop moving. It typically occurs in females and may occur during periods of extreme stress, which is sometimes why it may be called 'broken heart syndrome'. Patients are sometimes very unwell at the time of diagnosis and it may be mistaken for having a heart attack with chest pain and shortness of breath.  However, the heart usually recovers.

What Investigations Are Required?

The key test is an echocardiogram (ultrasound) which is the main test used to diagnose cardiomyopathies.  You will also need an electrocardiogram (ECG) to measure the electrical signals in the heart.  You are also likely to need a cardiac MRI scan which looks for abnormalities (often scarring) in the tissue of the heart.  If narrowings of the arteries supplying the heart are suspected then a CT scan of the heart may also be required. 

Depending on symptoms you may also need a 24-hour ECG to monitor your heart rhythm.  In some cases it may be suspected that you have a genetic cause of your cardiomyopathy (if other family members have been affected) and in this situation doing genetic testing may be useful but this is a very specialist area.

Cardiomyopathy Treatments

The treatment for a cardiomyopathy depends on the type.  Often the treatment is with diuretics (tablets to increase urine output and remove extra fluid) and medications such as ACE inhibitors and beta-blockers.  You may also need anticoagulants to thin the blood and lower the risk of blood clots.

In some cases your expert Consultant Cardioloigst may discuss a pacemaker, particularly if your electrocardiogram (ECG) shows that the electrical signal takes a long time to travel around your heart.  You may also be recommended to have an implantable cardioverter-defibrillator (ICD) inserted.  This monitors your heart rhythm and gives you an electrical shock to reset the rhythm if it detects a harmful rhythm.

In some cases, it may be felt that a problem with the heart rhythm is contributing to a patient’s symptoms or problems with the function of their heart.  For these patients, an ablation procedure may be offered to try and restore the normal rhythm of the heart.

Occasionally you may need surgical treatments, such as removing excess heart tissue from a thickened heart muscle in hypertrophic cardiomyopathy or treating leaking valves, but this is only useful in a small number of patients. For some people whose problem is limited to severe heart disease, (without problems in other parts of the body) they may be offered testing to identify if they would be suitable for heart transplant.

Schedule an appointment

Cardiomyopathies are complex conditions requiring careful evaluation by an expert Consultant Cardiologist.  Our consultants are available for consultation on any day of the week and weekends as well.