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Familial progressive cardiac conduction defect

^ http://www.orpha.net/ORDO/Orphanet_871


Familial progressive cardiac conduction defect (PCCD) is a hereditary cardiac conduction disorder that may progress to complete atrioventricular (AV) block. The disease is either asymptomatic or manifests as dyspnea, dizziness, syncope, abdominal pain, heart failure or sudden death.

Synonyms: Familial Lenègre disease, Hereditary bundle branch defect, Familial Lev-Lenègre disease, Familial PCCD, Familial progressive heart block, Familial Lev disease

Term info

database cross reference
  • UMLS:C1879286 (E (exact mapping (the terms and the concepts are equivalent)))
  • ICD-10:I45.8 (NTBT (narrower term maps to a broader term))
  • ICD-10:I45.8 (Attributed)
  • OMIM:115080 (NTBT (narrower term maps to a broader term))
  • OMIM:113900 (E (exact mapping (the terms and the concepts are equivalent)))
  • OMIM:140400 (BTNT (broader term maps to a narrower term))
  • OMIM:612838 (NTBT (narrower term maps to a broader term))
  • OMIM:604559 (BTNT (broader term maps to a narrower term))
hasDbXref

OMIM:115080, OMIM:604559, OMIM:113900, UMLS:C1879286, ICD-10:I45.8, OMIM:140400, OMIM:612838

notation

ORPHA:871