Brugada syndrome


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Brugada syndrome

A family of autosomal dominant conditions which manifest themselves in young individuals—especially from southeast Asia—with no known heart problems or defects, who suffer sudden cardiac death or aborted sudden cardiac death.
 
EKG
Right bundle branch block; persistent ST-segment elevation in V1 to V3, unexplained by electrolyte disturbances; ischaemia; structural heart disease.
 
Management
Implantable defibrillator.

Brugada syndromes
Type—MIM Number—Defective gene    
Brugada-1—601144—SCN5A      
Brugada-2—611778—GPD1L-611778
Brugada-3—114205—CACNA1C-
Brugada-4—600003—CACNB2
Brugada-5—604433—KCNE3
Brugada-6—600235—SCN1B

Brugada syndrome

Cardiology A condition in which people with no known heart problems or defects suffer sudden cardiac death or aborted sudden cardiac death EKG Right bundle branch block, persistent ST-segment elevation in V1 to V3 unexplained by electrolyte disturbances, ischemia, structural heart disease Treatment Implantable defibrillator. See Long Q-T syndrome.

Brugada syndrome

(broo-gŏd′ă)
A rare hereditary syndrome, occasionally autosomal dominant, marked by right bundle branch block, S-T segment elevation in the right precordial leads of the ECG, and a high risk of sudden death from ventricular arrhythmias.
References in periodicals archive ?
Propofol infusion syndrome and Brugada syndrome electrocardiographic phenocopy.
General anesthesia for a patient with Brugada syndrome.
Dr Sanjay Sharma, professor of clinical cardiology at St George's University Hospital, London, says: "One in 2,000 people have Brugada syndrome but the chance of you dying is 0.
Primary electrical diseases such as long QT syndrome, Brugada syndrome, short QT syndrome and catecholaminergic polymorphic ventricular tachycardia are inherited disorders with a strong genetic basis (5).
2,4) Differential diagnoses of Osborn waves include hypothermia, hypercalcemia, early repolarization, and Brugada syndrome.
Separately, we are very excited about the strong growth of Familion(TM), our proprietary genetic test to identify inherited forms of Long QT Syndrome and Brugada Syndrome, cardiac channelopathies that create serious risks in families with these genetic mutations.
More than 80 mutations associated with the Brugada syndrome have been identified (9) since the first indication of a genetic basis in 1998 (10).
Brugada syndrome, first identified in 1998, is a genetic disorder of the heart's electrical system.
The specific mechanism causing the differences between LQTS and Brugada syndrome is not known.
Effective April 1, 2012, the MTWA Test will be reimbursed for patients who are considered at risk for lethal arrhythmias including, but not limited to, patients with a history of heart attack, cardiomyopathy and Brugada syndrome.
Brugada syndrome, a cause of sudden cardiac death, is an inherited condition affecting around one in 2,000 people.