Cardiomyopathy

John Taylor died suddenly and unexpectedly. The post mortem revealed that he had died from cardiomyopathy, an enlarged heart. We cannot be sure if the condition was genetic, or viral. The doctors said that nothing could have been done to save John when his heart suddenly failed, but if the condition had been detected in advance then something might have been done to prolong his life.

The John Taylor foundation for young athletes was set up with the aims of providing awareness of the condition and to help put screening of athletes into place.


The following notes on cardiomyopathy by Dr. Phil Wallace are reproduced by kind permission of the Fellrunner magazine.

What is cardiomyopathy?
Cardiomyopathy is a heart muscle disease that can affect males and females of any ethnic origin. At the present time over 200,000 people in the UK are believed to have the condition. Although it is incurable, it can be treated with medication, pacing devices and surgery where appropriate.
There are three main types of cardiomyopathy:-

  • Hypertrophic cardiomyopathy
  • Dilated cardiomyopathy
  • Arrhythmogenic right ventricular cardiomyopathy

In cardiomyopathy the muscle of the heart is abnormal in the absence of an apparent cause. Excessive thickening of the heart muscle may occur (hypertrophy literally means “to thicken”). Heart muscle may thicken in any case in a normal individual as a normal physiological response to prolonged athletic training – the so-called “athlete’s heart”. The structure and function of such hearts is absoluteley normal; intense training in the absence of underlying heart disease and/or other medical conditions such as viral illnesses will not pose a threat to health.
Unfortunately, sudden death does occur in young, fit adults from time to time and a percentage of these will have hypertrophic cardiomyopathy. The cause is not yet known. In the majority of cases the condition is inherited, in others there is either no evidence of inheritance or there is insufficient information about the individual’s family to assess inheritance.

How does hypertrophic cardiomyopathy affect the heart?
The walls of the heart are made of specialised muscle known as myocardium. It is this part of the heart which is abnormal in cardiomyopathy. Every heartbeat in a normal heart results from an electrical signal starting at the top and passing down through the heart – the contraction of the heart follows the same course. The abnormality of the heart muscle in hypertrophic cardiomyopathy can sometimes interfere with this normal electrical activity. In abnormal segments of the heart the electrical signal may become unstable as it crosses areas of scarring and disarrayed cells. This in turn can lead to disorganised electrical impulses that generate fast or erratic heart rhythms. Some of these rhythms can be life-threatening.
When does hypertrophic cardiomyopathy develop?
Rarely, it is present at birth. However, hypertrophy more commonly develops in association with growth and is usually apparent in the late teens and early twenties. Children and adolescents with the condition are usually identified when family screening is performed after an adult in the family is found to be affected. Of these adults 50% will have experienced symptoms; in the remainder the diagnosis is made during family screening or following the detection of a murmur or an abnormality on routine electrocardiography/echocardiography.
What symptoms does hypertrophic cardiomyopathy cause?
There is no particular symptom or complaint which is unique to hypertrophic cardiomyopathy. The reason for the onset of symptoms is often not clear, although they may occur at any stage in a person’s life even though the condition may have been present for some time.
Symptoms may include:-

  • Inappropriate shortness of breath.
  • Chest pain.
  • Palpitations (awareness of the heart beating irregularly or very fast).
  • Light-headedness and blackouts.

Hypertrophic cardiomyopathy may be suspected because of symptoms, a murmur or an abnormal ECG. An individual may present any of the symptoms described above but, because such symptoms could be caused by a large number of other conditions, further tests are necessary.

What is the outcome for affected persons?
The severity of symptoms and the risk of complications vary greatly between patients. It should be emphasised that many people never have any serious problems related to this condition. Each person, however, must be carefully assessed and advised by a cardiologist.
Hypertrophic cardiomyopathy is not caused by athletic training.
 

Some useful WWW Links

www.cardiomyopathy.org The Cardiomyopathy Association

www.bhf.org.uk The British Heart Foundation

www.c-r-y.org.uk CRY – Cardiac Risk in the Young