Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease of the heart muscle. In this disease, fatty fibrous tissue replaces normal heart muscle. This interrupts normal electrical signals in the heart and may cause irregular and potentially life-threatening heart rhythms. The heart also becomes weaker over time leading to heart failure. It was previously called arrhythmogenic right ventricular dysplasia (or ARVD).
ARVC typically begins in a small part of the right ventricle. Over time, the disease slowly affects more of the right ventricle. Sometimes the left ventricle is affected, too. This can lead to abnormal heart rhythms, and sometimes sudden death. Over time, the heart can’t pump as much blood forward as it normally would. Blood backs up in the circulatory system causing fluid to build up in the soft tissues or the lungs. This can lead to many symptoms such as swelling (edema) and shortness of breath. This is called heart failure.
ARVC is a fairly rare genetic disease. It usually starts between the ages of 10 and 50 years old. The severity of the disease varies a great deal between people.
What causes ARVC?
ARVC results from a genetic defect. Genes are part of your DNA, the material passed down from parents to children. This genetic defect leads to the replacement of normal heart tissue with fatty or fibrous tissue. How this happens is not yet clear.
Most cases of ARVC are from an autosomal dominant inheritance. This means you need an abnormal gene from only one of your parents to have it. Still, even if you have an abnormal gene, you might not develop any major symptoms from ARVC. Researchers are still trying to understand what other factors may increase disease severity.
What are the symptoms of ARVC?
Symptoms of ARVC tend to get worse over time as the disease affects more of the heart. Some people with ARVC show no symptoms. And others may have more severe symptoms. These may include:
- Fainting
- Heart palpitations with unpleasant awareness of the heartbeat
- Dizziness
- Shortness of breath with exertion or when lying down
- Chest pain
- Fatigue
- Swelling in the legs and other areas
- Persistent cough
- Sudden death due to an abnormal heart rhythm
Heart palpitations and fainting are common early symptoms. Unfortunately, sudden death is also sometimes the first symptom of ARVC. The other symptoms tend to come on more gradually.