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Familial isolated arrhythmogenic right ventricular dysplasia

Disease definition

Familial isolated arrhythmogenic right ventricular dysplasia (ARVC) is the familial autosomal dominant form of ARVC (see this term), a heart muscle disease characterized by life-threatening ventricular arrhythmias with left bundle branch block configuration that may manifest with palpitations, ventricular tachycardia, syncope and sudden fatal attacks, and that is due to dystrophy and fibro-fatty replacement of the right ventricular myocardium that may lead to right ventricular aneurysms.

ORPHA:217656

  • Synonym(s):
    • Familial isolated ARVC
    • Familial isolated ARVD
    • Familial isolated arrhythmogenic right ventricular cardiomyopathy
    • Familial isolated arrhythmogenic ventricular cardiomyopathy
    • Familial isolated arrhythmogenic ventricular dysplasia
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: -
  • ICD-10: I42.8
  • OMIM: 107970  600996  602086  602087  604400  604401  607450  609040  610193  610476  611528  615616
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

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