Print friendly version
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.
On this page
Genetics are thought to play a large role in causing Lenegre disease. Several alterations in the "SCN5A" gene have been found in association with familial heart block. It is thought that a single gene mutation in combination with aging leads to the progressive heart block seen in Lenegre disease. Genetic risk for familial heart block in children of affected individuals can be as high as 50%. Genetic testing for Lenegre disease is available. For further information on testing we recommend you speak with your healthcare provider or a genetic professional.
Last updated: 5/13/2013
- Probst V et al.,. Haploinsufficiency in combination with aging causes SCN5A-linked hereditary Lenègre disease. J Am Coll Cardiol. 2003 Feb 19;41(4):643-52; http://www.ncbi.nlm.nih.gov/pubmed/?term=12598077. Accessed 5/13/2013.
- Sauer WH. Etiology of atrioventricular block. In: Basow, DS. UpToDate. Waltham, MA: UpToDate; 2013;