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Familial progressive cardiac conduction defect
Other Names for this Disease
- Familial Lenègre disease
- Familial Lev disease
- Familial Lev-Lenègre disease
- Familial PCCD
- Familial progressive heart block
- Progressive familial heart block type 1A
- Progressive familial heart block type 1B
- Progressive familial heart block type 2
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What is familial progressive cardiac conduction defect?
Familial progressive cardiac conduction defect (PCCD) is a is a cardiac (heart) conduction disorder that may progress to complete heart block. Affected people may not have any symptoms, or the condition may cause shortness of breath, dizziness, fainting, abdominal pain, heart failure, or sudden death. Mutations in several genes, including the SCN5A, SCN1B and TRPM4 genes, can cause PCCD. Several other genes may be the cause when PCCD occurs with congenital heart disease. Familial PCCD is usually inherited in an autosomal dominant manner. However, not all people that have the mutated gene will have the condition; in those that do, symptoms and severity can vary (known as reduced penetrance and variable expressivity). Autosomal recessive inheritance and sporadic cases have been reported, but are rare. Treatment includes implantation of a pacemaker.
Last updated: 3/20/2014
- Vincent Probst. Familial progressive cardiac conduction defect. Orphanet. December, 2013; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=871. Accessed 3/18/2014.