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(also called arrhythmogenic right ventricular cardiomyopathy)
ARVD is a rare form of cardiomyopathy in which the heart muscle of the right ventricle (RV) is replaced by fat and/or fibrous tissue. The right ventricle is dilated and contracts poorly. As a result, the ability of the heart to pump blood is usually weakened. Patients with ARVD often have arrhythmias (abnormal heart rhythms), which can increase the risk of sudden cardiac arrest or death.
The cause of ARVD is unknown. It occurs in about 1 in 5,000 people. ARVD can occur with no family history, although often it runs in families. A family history of ARVD is present in at least 30 to 50 percent of cases. Therefore, it is recommended that all first- and second-degree family members (parents, siblings, children, grandchildren, uncle, aunt, nephew, niece) be evaluated carefully for this form of cardiomyopathy, even in the absence of symptoms.
Researchers have found two patterns of inheritance for ARVD:
Not all the specific genes have been identified as a cause for ARVD. Researchers are attempting to identify the specific gene mutations and locations of chromosomes associated with ARVD.
ARVD may also be related to non-genetic causes such as congenital abnormalities (affecting the right ventricle), viral or inflammatory myocarditis.
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