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The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.<cite>McKenna1994</cite> | 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 | 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 mutations 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. | One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance. | ||
'''Diagnosis''' ARVC is a difficult diagnosis to make. Therefore, the European Society of Cardiology has created a list of diagnostic criteria for the diagnosis of ARVC<cite>#McKenna1994</cite> (see table). An [arvc.ca/pdg/public.php?rep=arvc_cri online calculator] can help in assessing the risk in an individual patient. In 2009 these criteria were updated<cite>cox</cite><cite>Crit2010</cite>, see the table below. | '''Diagnosis''' ARVC is a difficult diagnosis to make. Therefore, the European Society of Cardiology has created a list of diagnostic criteria for the diagnosis of ARVC<cite>#McKenna1994</cite> (see table). An [http://www.arvc.ca/pdg/public.php?rep=arvc_cri online calculator] can help in assessing the risk in an individual patient. In 2009 these criteria were updated<cite>cox</cite><cite>Crit2010</cite>, see the table below. | ||
'''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite> | '''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite> |