Brugada syndrome

Also known as: Lai Tai; Sudden unexplained nocturnal death syndrome (SUNDS)

ECG pattern of coved ST elevation in right precordial leads with risk of ventricular fibrillation in a structurally normal heart.

Medical specialty: Cardiology
Subspecialty: Cardiology / Cardiac electrophysiology
Sex & age distribution: Male: affected, about 8–10 times more common; Female: affected, less often; Children: rare, usually onset 30s–40s; fever-triggered events in children reported
Gene(s) involved: ABCC9, CACNA1C, CACNB2, FGF12, GPD1L, HCN4, KCND3, KCNE3, KCNE5, KCNJ8, PKP2, RANGRF, SCN1B, SCN2B, SCN3B, SCN5A, SEMA3A, SLMAP, TRPM4

Etiology & mechanism

Reduced sodium current or imbalance of transient outward current causes epicardial conduction heterogeneity in the right ventricular outflow tract.

Gene(s) involved (19)

ABCC9CACNA1CCACNB2FGF12GPD1LHCN4KCND3KCNE3KCNE5KCNJ8PKP2RANGRFSCN1BSCN2BSCN3BSCN5ASEMA3ASLMAPTRPM4

Symptoms & signs (7)

coved st elevation in v1–v3fever-triggered eventsnocturnal agonal respirationpalpitationssudden cardiac deathsyncopeventricular fibrillation

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