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Familial progressive cardiac conduction defect

Disease definition

A genetic cardiac rhythm disease 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.


Classification level: Disorder
  • Synonym(s):
    • Familial Lenègre disease
    • Familial Lev disease
    • Familial Lev-Lenègre disease
    • Familial PCCD
    • Familial progressive heart block
    • Hereditary bundle branch defect
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: I45.8
  • ICD-11: BC63.Y
  • OMIM: 113900  115080  140400  604559  612838
  • UMLS: C1879286
  • MeSH: C566873
  • GARD: 10005
  • MedDRA: -

Detailed information


Genetic Testing

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)
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