Terminology Service for NFDI4Health
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familial isolated arrhythmogenic right ventricular dysplasia

Go to external page http://purl.obolibrary.org/obo/MONDO_0016342

Familial isolated arrhythmogenic right ventricular dysplasia (ARVC) is the familial autosomal dominant form of ARVC, 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. [ ]

Term info

Label

familial isolated arrhythmogenic right ventricular dysplasia

Synonyms
  • familial isolated ARVC
  • familial isolated ARVD
  • familial isolated arrhythmogenic right ventricular cardiomyopathy
  • familial isolated arrhythmogenic right ventricular dysplasia
  • familial isolated arrhythmogenic ventricular cardiomyopathy
  • familial isolated arrhythmogenic ventricular dysplasia
database cross reference
Subsets

ordo_disease

exactMatch

http://purl.obolibrary.org/obo/Orphanet_217656, http://linkedlifedata.com/resource/umls/id/CN226907, http://linkedlifedata.com/resource/umls/id/C4274968, http://identifiers.org/snomedct/715865008, https://omim.org/phenotypicSeries/PS107970

id

MONDO:0016342

narrowMatch

https://icd.codes/icd10cm/I42.8