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Progressive cardiac conduction defect
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Progressive cardiac conduction defect | |
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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 |
Progressive cardiac conduction defect (PCCD) is a rare inherited degenerative disease of a heart's electrical conduction system. Over time PCCD can lead to a complete heart block and might require a pacemaker implantation to treat it.
Genetics
Most commonly PCCD is inherited as a autosomal dominant defect and is caused by mutations of chromosome 19q13.3 and in SCN5A, SCN1B and TRPM4 genes.