Progressive Cardiac Conduction Disease (PCCD) refers to a heterogeneous group of cardiac disease. Isolated PCCD involves a structurally normal heart.
Features of isolated PCCD:
(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 normal 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 isolated PCCD:
(1) SCN5A
(2) TRPM4 (transient receptor potential channel, subfamily M, member 4)
