Cardiomyopathy and athlete's heart

At the inherited cardiac conditions service we treat the following conditions related to the heart’s muscle tissue:
• Hypertrophic cardiomyopathy
• Arrhythmogenic right ventricular dysplasia
• Dilated cardiomyopathy
• Athlete’s heart

Hypertrophic cardiomyopathy
Hypertrophic cardiomyopathy is a genetic condition that causes excessive thickening of the heart muscle, which can increase the risk of dangerous heart rhythms and sudden death. It affects up to one in 500 people.

Man having an ultrasound scan (echocardiogram) of his heartOur team includes specialists in cardiology, the electrical function of the heart and genetics, who work together to evaluate each patient and develop the best treatment plan. This team approach is particularly effective in the treatment of hypertrophic cardiomyopathy, which is a complex disease that can be caused by a variety of genetic abnormalities.

If a child has one parent with hypertrophic cardiomyopathy, they have a 50:50 chance of inheriting the condition – this is known as an ‘autosomal dominant’ condition. Not everyone who inherits the condition will develop symptoms. This means it is important to test the family members of a patient. Our specialists are experts in these issues and can recommend which family members should be tested.

Treatment
Although many patients with hypertrophic cardiomyopathy need little treatment and have a normal lifespan, certain patients have an increased risk of dangerous heart rhythms and sudden death. They may need to be fitted with an implantable cardiac defibrillator (ICD). An ICD is a small battery-powered device that can detect an abnormal heart rhythm and correct it by producing a small jolt of electricity.

Our cardiologists are world leaders in electrical problems of the heart and have substantial experience in the fitting of ICDs and aftercare.

Man having an ECG while running on a treadmillArrhythmogenic right ventricular dysplasia
Arrhythmogenic right ventricular dysplasia (ARVD) is a relatively rare form of cardiomyopathy, affecting up to one in 5,000 people. In this condition the heart muscle of the right side of the heart (right ventricle) is replaced by fat and/or scar tissue. It is usually diagnosed in people less than 40 years old. Patients with ARVD often have abnormal heart rhythms (arrhythmias), which may increase the risk of a sudden heart attack or death.

Our world-class team of experts is able to manage the complications of ARVD, such as abnormal heart rhythms or heart failure, if these occur.

Treatment
Medication to control abnormal heart rhythms is the most frequently used treatment in ARVD. Patients who are thought to be at risk for sudden death are usually fitted with an implantable cardiac defibrillator (ICD).

Dilated cardiomyopathy
Dilated cardiomyopathy is a condition in which the heart becomes enlarged due to thinning, and often reduced function, of the heart muscle. Around 40 percent of cases are inherited. Other causes include heart disease, high blood pressure and excessive alcohol intake. Therefore family screening is recommended when no clear underlying cause is found.

Treatment
The management of dilated cardiomyopathy is similar to that of heart failure from other causes, and focuses on drug treatment that aims to alleviate symptoms and improve heart function. Some patients will be fitted with a sophisticated pacemaker (biventricular pacemakers) or defibrillator.

Athlete’s heart
Vigorous athletic training can occasionally lead to detectable changes in the heart, namely an enhanced, but normal, thickening of the heart muscle. The condition is benign, reversible and not inherited.

‘Athlete’s heart’ can show as abnormalities on tests of the heart such as an electrocardiogram (ECG) and occasionally an ultrasound scan (echocardiogram). These findings are not predictable and can occur more commonly in some ethnic groups than others.

Where confusion can arise is in telling apart this normal response to exercise from the abnormal conditions that these changes sometimes mimic, namely cardiomyopathy (see above). When screening athletes it is therefore important to have a cardiologist with expertise in both cardiomyopathy and athlete’s heart and access to specialist imaging techniques such as cardiac magnetic resonance imaging (MRI).