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Brugada syndrome

Diseases of the Circulatory System

Brugada syndrome is an inherited disorder of the heart's electrical system, caused most often by mutations in the SCN5A gene that governs cardiac sodium channels. The condition disrupts normal electrical signaling in the heart muscle, raising the risk of dangerous fast heart rhythms and sudden cardiac death, often in people who appear otherwise healthy. Roughly one in four affected individuals inherits the mutation from a parent in an autosomal dominant pattern, while others develop it from a new mutation or as a reaction to certain medications. Episodes of fainting or cardiac arrest often strike during sleep or rest, and can be triggered by fever, heavy alcohol use, or drugs such as cocaine. The condition was first linked to sudden cardiac death in 1989 by the Italian cardiologists Bruno Martini and Antonio Nava, and it was formally described and named in 1992 by the Spanish brothers Pedro and Josep Brugada, whose family name it carries. Diagnosis relies on a distinctive electrocardiogram pattern, and treatment for those at high risk typically involves an implantable cardioverter defibrillator.

Facts
Classification
Body System
Cardiovascular System 1
ICD-10 Chapter
IX: Diseases of the Circulatory System 1
Communicable or Non-communicable
Non-Communicable 1
Causative Agent
Causative Agent (category)
Genetic / Hereditary 1
First Described
1989 1
Sources
1. Brugada syndrome (Wikipedia)
  • lead sentence 1
    Brugada syndrome (BrS) is a genetic disorder in which the electrical activity of the heart is abnormal due to channelopathy.
  • Lead: disease category
    Brugada syndrome (BrS) is a genetic disorder in which the electrical activity of the heart is abnormal due to channelopathy.
  • Article text
    It was first described by Andrea Nava and Bortolo Martini, in Padova, in 1989; it is named after Pedro and Josep Brugada, two Spanish cardiologists, who described the condition in 1992.
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