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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Brugada syndrome


Other Names for this Disease

  • Right bundle branch block, ST segment elevation, and sudden death syndrome
  • Sudden unexpected nocturnal death syndrome
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Inheritance

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How is Brugada syndrome inherited?

Brugada syndrome is inherited in an autosomal dominant manner. This means that having one mutated copy of the responsible gene in each cell is enough to cause signs or symptoms. Almost all people with Brugada syndrome have one parent with the condition. In about 1% of cases, an affected person has a new mutation in the responsible gene and has no family history of the condition.[1]

Unsymptomatic parents of an affected person should be evaluated with electrocardiography, and any family history of sudden death should be discussed. If a mutation is identified in the affected person, genetic testing of the parents is recommended. A family history may appear to be negative due to reduced penetrance, death of a parent before symptoms start, or late onset of symptoms in an affected parent.[1]

Each child of an affected person has a 50% chance to inherit the mutated gene. The risk to other family members depends on whether a parent carries the mutation and is affected.[1]
Last updated: 4/22/2014

References
  1. Ramon Brugada, Oscar Campuzano, Pedro Brugada, Josep Brugada, and Kui Hong. Brugada Syndrome. GeneReviews. April 10, 2014; http://www.ncbi.nlm.nih.gov/books/NBK1517/. Accessed 4/23/2014.


Other Names for this Disease
  • Right bundle branch block, ST segment elevation, and sudden death syndrome
  • Sudden unexpected nocturnal death syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.