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What should you do if you are diagnosed with HCM? If your tests prove positive your specialist will advise you on your lifestyle and you will probably be advised not to participate in continuous strenuous activities e.g. competitive sports. Most of the time the condition should not significantly interfere with your lifestyle and can be controlled by drugs if necessary. It will be necessary for you to have at least annual check-ups. However, the severity of the disease varies from person to person and even if you have been diagnosed with HCM you may not necessarily present any symptoms and can live a normal life. Since the disease runs in families, all immediate blood relatives of affected patients have to be screened with an ECG and an ECHO.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) The estimated prevalence of arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia (ARVD), in the general population ranges from 1 in 1,000 to 1 in 5,000 depending on the definition of the condition. It was first recognised in the late 1970s. We think that even more information regarding ARVC will be available in the coming years, to improve our understanding of the condition. The disease affects men and women equally and has been recognised in people of diverse ethnic backgrounds. It tends to develop in early adulthood but can occasionally affect children.

ARVC is caused by a defect in the ‘glue’ that holds the muscle cells of the heart together, working as a unit. When stretched the ‘glue’ breaks down, the muscle cells separate and some die. The body then tries to repair this, resulting in replacement of the normal heart muscle cells by scar and fat tissue. This may only involve small areas of the right ventricle but may progress with time and may become more widespread and even involve the left ventricle. Some forms only affect the left ventricle.

What are the symptoms? Often you may have no symptoms at all. You may feel palpitations (feeling the pulse racing or the heart pounding erratically or rapidly) which is the most common symptom. This may also be associated with light-headedness or a blackout. Unlike most cardiomyopathies, shortness of breath and chest pains are unusual symptoms and you may only experience these very late on in the condition, if at all.

How is it diagnosed? The diagnosis can be extremely difficult and usually requires several tests undertaken by a specialist as there is no single ‘diagnostic’ test. In the first instance a detailed history and examination is required. Most of the tests are painless and non-invasive and include an ECG, signal average ECG and an ultrasound scan (ECHO) (we describe these tests on pages 26-27). The ECG can often pick up an abnormality but the signs on an ECHO can be very subtle in the early stages of the

Cardiac Risk in the Young 16

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