Arrhythmogenic Right Ventricular Cardiomyopathy: Difference between revisions

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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 chromosomal defects have been described, most often with autosomal dominant inheritance.  
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>

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