progressive familial heart block

Term ID

DOID:0111073

Synonyms

• familial Lenegre disease
• familial Lev disease
• familial Lev-Lenegre disease
• familial PCCD
• familial progressive heart block
• hereditary bundle branch defect
• PFHB

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. https://www.ncbi.nlm.nih.gov/pubmed/897853

References

• MIM:115080
• MIM:PS113900
• ORDO:871

Ontology

Human Disease   ( DOID:0111073 )