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

More general terms

• familial heart block

Database

• OMIM: https://mirror.omim.org/entry/113900