ARVC is caused by a defect in the 'glue' that holds the muscle cells of the heart together. As the 'glue' breaks down, the muscle cells separate and some die. The body then tries to repair this by replacing the normal heart muscle cells with scar and fat tissue which do not conduct electrical signals and contract in the normal way. This allows abnormal 'short-circuits' to develop, which cause the heart rhythm abnormalities that define the condition.
The most common symptom is palpitations. Palpitations can occur at rest but are often triggered by physical activity. They may be associated with chest pain, light-headedness or blackouts. ARVC is also a recognised cause of sudden cardiac death. If the right ventricle becomes significantly weakened it can cause breathlessness and swelling of the legs and abdomen, although this is relatively uncommon and tends to affect those who have had the condition for several years or longer.
Detailed record of symptoms, medical and family background and a physical examination
ECG (including a signal average ECG) and an ultrasound scan of the heart (echocardiogram, or echo)
Magnetic resonance imaging (MRI) scan
Exercise ECG and 24-hour ECG to record any palpitations or abnormal heart rhythms
If a specific genetic mutation has been identified as the cause of ARVC in an individual,
family members can be tested for this same mutation with a blood test.
Lifestyle modifications such as not participate in competitive, strenuous physical activities
since this can be a trigger for dangerous heart rhythm disturbances
Medication including beta-blockers, sotalol and amiodarone
Ablation : the particular area of the heart causing the abnormal rhythm and this area is
cauterised – or burnt. However, new problematic areas of the heart can develop over time
Implantable cardiac defibrillator (ICD)