Arrhythmogenic right ventricular cardiomyopathy, previously known as arrhythmogenic right ventricular dysplasia, is a condition in which part of the heart muscle is replaced by fibrous tissue and fat. The affected area of heart muscle is in the wall of the right ventricle, or right lower chamber, of the heart, and the left ventricle may also be involved. Loss of heart muscle means that the heart pumps less effectively and can interfere with the passage of electrical impulses that make the heart contract, leading to an irregular heartbeat. Sometimes, without any signs developing first, the condition causes a person to die suddenly. Arrhythmogenic right ventricular cardiomyopathy is one of the most frequent causes of sudden death in younger people.
There are a number of types of cardiomyopathy, and these are often inherited heart diseases, occurring in several members of the same family. Some cardiomyopathies have known causes, but it is not understood why arrhythmogenic right ventricular cardiomyopathy develops. Young adults are most frequently affected by the condition, particularly males below the age of 40.
Symptoms and signs of arrhythmogenic right ventricular cardiomyopathy may include light-headedness and shortness of breath. Fainting and palpitations may be experienced, and the ankles or belly might swell. As the disease progresses through different stages, symptoms may vary and diagnosis can be difficult. In the early stages, there may be no obvious signs, while in the later stages heart failure may develop. If the disease is diagnosed early, treatment may be given to help the heart function more normally to prevent irregular heartbeats and lower the risk of sudden death.
Treatments for arrhythmogenic right ventricular cardiomyopathy include drugs which regulate abnormal heart rhythms and techniques such as cardioversion and catheter ablation, which alter the electrical activity of the heart. Cardioversion uses electric shocks to normalize the heart's rhythm, while catheter ablation destroys areas of heart muscle which are giving out abnormal electrical signals and triggering irregular heart rates. For some people, feeling dizzy and passing out may be warning signs indicating an increased risk of sudden death.
A device known as an implantable cardioverter defibrillator, or ICD, may be fitted beneath the skin, with wires leading to the heart. If a potentially fatal abnormal rhythm develops, the ICD delivers a shock to the heart which restores a normal heartbeat. In cases where heart failure develops, drugs may be given to reduce the fluid load on the heart and improve its ability to act as a pump.
