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Familial isolated arrhythmogenic right ventricular dysplasia

Orpha number ORPHA217656
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 -
Inheritance
  • Autosomal dominant
Age of onset -
ICD-10
  • I42.8
OMIM
UMLS -
MeSH -
MedDRA -
SNOMED CT -

Summary

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.


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Detailed information

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