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Arrhythmogenic right ventricular cardiomyopathy

Disease definition

Arrhythmogenic right ventricular cardiomyopathy (ARVC) 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 a risk of sudden cardiac death.

ORPHA:247

  • Synonym(s):
    • ARVC
    • ARVD
    • Arrhythmogenic right ventricular dysplasia
  • Prevalence: 1-5 / 10 000
  • Inheritance: Autosomal dominant or Autosomal recessive 
  • Age of onset: Adolescent, Adult
  • ICD-10: I42.8
  • OMIM: -
  • UMLS: C0349788
  • MeSH: D019571
  • GARD: -
  • MedDRA: 10058093

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