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Diseases

Genetic and Rare Diseases Information Center (GARD)

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?

The genetic form of Brugada syndrome (not the acquired form) 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 a 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. Each child of an affected person has a 50% chance to inherit the mutated gene.[1]

Unsymptomatic parents of an affected person should be evaluated with electrocardiography, and any family history of sudden death should be discussed. If genetic testing reveals a mutation 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]
Last updated: 3/16/2016

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/.


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.