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 ?
Brugada Syndrome is an arrhythmic genetic syndrome inherited as autosomal dominant with incomplete penetrance and variable expressivity.
The Brugada syndrome is said to be extremely rare and takes time to be diagnosed.
The popular dad had been diagnosed with Brugada syndrome, which can cause irregular heart movements and seizures, in 2015 but was told by doctors he was a low-risk sufferer.
This result was consistent with inhibition of the Brugada wave and polymorphic ventricular tachycardia at the tissue level, suggesting that Wenxin Granule may be effective in the treatment of J-wave syndromes, including Brugada syndrome.
The prevalence of Brugada syndrome appears to be low in the general population.
Despite decades of research into the genetics of Mendelian cardiac disease, the genetic test yield for several ICCs has remained stubbornly low, particularly for important conditions like HCM (approximately 40%), DCM (approximately 30% at best) and Brugada syndrome (approximately 20% at best).
Ohgo T, Okamura H, Noda T, Satomi K, Suyama K, Kurita T (2007) Acute and chronic management in patients with Brugada syndrome associated with electrical storm of ventricular fibrillation.
Toward a unifying mechanism of the Brugada syndrome," Circulation: Arrhythmia and Electrophysiology, vol.
As a model of a primary rhythm disorder, Brugada syndrome (BrS may be considered as ~sensitized model~ for SCD and be relevant to the broad problem of SCD in the setting of common cardiac pathologies.
The ECG shows coved Type 1 ST segment elevation in keeping with a diagnosis of Brugada syndrome.
This patient's EKG showed a type 1 Brugada pattern (FIGURE 2, ARROWS), which strongly supported the diagnosis of Brugada syndrome (BS).