FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
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General Information
Term progressive familial heart block ID (Ontology) DOID:0111073 (Human Disease)
Definition A heart conduction disease characterized by autosomal dominant inheritance of a cardiac conduction defect that may progress to complete atrioventricular (AV) block and maybe asymptomatic of manifest as shortness of breath, dizziness, syncope, abdominal pain, heart failure or sudden death.
Also Known As "familial Lenegre disease" ; "familial Lev disease" ; "familial Lev-Lenegre disease" (for all, see Synonyms field below)
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  heart disease
   |__heart conduction disease
       |__progressive familial heart block  3 rec.
           |__progressive familial heart block type IA 2 rec.
           |__progressive familial heart block type IB 1 rec.
           |__progressive familial heart block type II
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Is a heart conduction disease
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Synonyms
  • "familial Lenegre disease" EXACT
    "familial Lev disease" EXACT
    "familial Lev-Lenegre disease" EXACT
    "familial PCCD" EXACT
    "familial progressive heart block" EXACT
    "hereditary bundle branch defect" EXACT
    "PFHB" EXACT OMO:0003012
Secondary IDs
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MIM:115080
MIM:PS113900
ORDO:871