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Arrhythmogenic right ventricular dysplasia

Orpha number ORPHA247
Synonym(s) ARVC
ARVD
Arrhythmogenic right ventricular cardiomyopathy
Prevalence 1-5 / 10 000
Inheritance Autosomal recessive
Autosomal dominant
Age of onset Adult
ICD-10
  • I42.8
ICD-O -
OMIM -
UMLS
  • C0349788
MeSH
  • D019571
MedDRA
  • 10058093

Summary

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a heart muscle disease that consists in progressive dystrophy of primarily the right ventricular myocardium with fibro-fatty replacement and ventricular dilation, and that is clinically characterized by ventricular arrhythmias. and progressive right ventricular or biventricular failure. ARCVD is a major cause of sudden death in the young and among athletes.

Expert reviewer(s)

  • Pr Cristina BASSO

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Detailed information

Summary information
Review article
  • EN (2007)
Clinical practice guidelines
  • EN (2013)
Guidance for genetic testing
  • EN (2013,pdf)
Clinical genetics review
  • EN (2014)
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