Progressive Cardiac Conduction Disease (PCCD) refers to a heterogeneous group of cardiac disease. Some cases of PCCD involve a structurally abnormal heart.


Features of PCCD with a structurally abnormal heart:

(1) The age of onset is < 50 years.

(2) The patient has progressive conduction abnormalities (AV block, RBBB, LBBB, etc).

(3) The patient has a structurally abnormal heart without evidence of skeletal myopathy.

(4) Most cases are familial.

(5) The conduction abnormalities cannot be better explained by another diagnosis.


Genes with mutations associated with PCCD and congenital heart disease:

(1) NKX2.5

(2) TBX5


Genes with mutations associated with PCCD and dilated cardiomyopathy:

(1) LMNA


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