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progressive familial heart block type 1A; Lenegre-Lev disease; progressive cardiac conduction defect (PCCD, PFHB1A)
Pathology:
- cardiac bundle branch disorder that may progress to complete heart block
- progressive alteration of cardiac conduction through the His-Purkinje system with right bundle branch block or left bundle branch block, leading to complete AV block
Genetics:
- autosomal dominant
- associated with defects in SCN5A
Clinical manifestations:
- complete AV block may lead to syncope & sudden death Special laboatory:
- right bundle branch block or left bundle branch block
- widening of QRS complexes
- complete AV block may occur
General
familial heart block
Database Correlations
OMIM 113900
References
OMIM :accession 113900