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familial isolated arrhythmogenic right ventricular dysplasia

^ 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. [ Orphanet:217656 ]

Term info

database cross reference
  • UMLS:C4274968 (MONDO:equivalentTo)
  • SCTID:715865008 (MONDO:equivalentTo)
  • UMLS:CN226907 (MONDO:equivalentTo)
  • OMIMPS:107970 (MONDO:equivalentTo)
  • Orphanet:217656 (MONDO:equivalentTo)
Subsets

ordo_disease

definition

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.

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

has exact synonym

familial isolated arrhythmogenic right ventricular cardiomyopathy, familial isolated ARVC, familial isolated arrhythmogenic ventricular dysplasia, familial isolated ARVD, familial isolated arrhythmogenic right ventricular dysplasia, familial isolated arrhythmogenic ventricular cardiomyopathy

id

MONDO:0016342