Arrhythmogenic Right Ventricular Dysplasia is a type of cardiomyopathy, a disease that affects the heart muscle, the myocardium, causing abnormal heart rhythms or arrhythmia. It is a condition that sets in when the right ventricle of the heart is affected, causing the muscle to be replaced by fat and fibrosis. This leads to arrhythmia. The change in heart rhythm makes it difficult for the heart to pump blood. It can also disrupt the flow of blood to the myocardium and cause chest pain.
Death happens when the heart can not pump enough blood to the myocardium and to the lungs. However, it does not often lead to cardiac deaths but it accounts for one-fifth of the cardiac deaths in people under 35 years of age. Arrhythmogenic Right Ventricular Dysplasia affects both men and women.
It is also known by other names such as:
- Arrhythmogenic right ventricular cardiomyopathy
- Arrythmogenic right ventricular cardiomyopathy of Naxos
- Naxos disease
- Naxos syndrome
- Diffuse non-epidermolytic palmoplantar keratoderma with woolly hair and cardiomyopathy
Arrhythmogenic Right Ventricular Dysplasia is developed either through inheritance when an affected parent passes on a certain gene change to a child. It could also result from the infection of the myocardium.
There is no single test that can make the diagnosis and it is often to difficult to diagnose the condition. The diagnosis of Arrhythmogenic Right Ventricular Dysplasia can be done by a number of cardiac tests including ECG, an exercise stress test, a cardiac MRI and a 24-hour Holter monitor. Additional tests include an EP (Electro Physiology) study, right ventriculogram, biopsy, a CT scan and MUGA scan.
The symptoms of Arrhythmogenic Right Ventricular Dysplasia come as a result of the abnormal heart rhythms, which could be observed in a healthy person as well. Some of the commonly observed symptoms include palpitations, lightheadedness, nausea, heart racing, heart fluttering and fainting. Symptoms could also be that of a heart failure which includes swelling of feet, extreme fatigue, shortness of breath and so on.
Arrhythmogenic Right Ventricular Dysplasia is treated by controlling the arrhythmia. This is done either by medication or by attaching a device to the heart which will keep the heart rhythm in check. However, these methods may not always work since the disease is progressive in nature.
If the symptoms of heart failure show up, it is treated with medications. This includes ACE inhibitors that could make it easier for the heart to pump blood. Also doctors suggest diuretics which regulate symptoms of heart failure.