The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.<cite>McKenna1994</cite>
ARVC is a progressive disease. The '''incidence''' is estimated to be 1:3.000-1:10.000. Manifestations are usually seen in teenagers. Although the diagnosis is more often made in athletes, sports are not thought to have a causative relationship with the disease. ARVD can occur in families; more than 9 different
chromosomal defects have been described, most often with autosomal dominant inheritance.
One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.