Progressive cardiac conduction defect

Progressive cardiac conduction defect (PCCD) is a hereditary cardiac condition marked by a progressive delay in impulse conduction via the His-Purkinje system, resulting in right or left bundle branch block (RBBB or LBBB), syncope, and occasionally sudden cardiac death.^[1]

Progressive cardiac conduction defect
Other names	Familial Lenègre disease, Familial Lev disease, Familial Lev-Lenègre disease, Familial PCCD, Familial progressive heart block, Progressive familial heart block, Hereditary bundle branch defect
Specialty	Cardiology

Cause

Diagnosis

When progressive conduction abnormalities in people under 50 with structurally normal hearts are present but skeletal myopathies are absent, progressive cardiac conduction defect is primarily diagnosed, particularly if there is a family history of PCCD.^[2]

Treatment

At the moment, implanting an implantable pacemaker is the only proven treatment for PCCD, regardless of the underlying cause.^[3]