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Brugada syndrome 1(BRGDA1)

MedGen UID:
Concept ID:
Disease or Syndrome
Synonyms: BRGDA1; Right bundle branch block, ST segment elevation, and sudden death syndrome; SCN5A-Related Brugada Syndrome
Gene (location): SCN5A (3p22.2)
Monarch Initiative: MONDO:0011001
OMIM®: 601144

Disease characteristics

Excerpted from the GeneReview: Brugada Syndrome
Brugada syndrome is characterized by cardiac conduction abnormalities (ST segment abnormalities in leads V1-V3 on EKG and a high risk for ventricular arrhythmias) that can result in sudden death. Brugada syndrome presents primarily during adulthood, although age at diagnosis may range from infancy to late adulthood. The mean age of sudden death is approximately 40 years. Clinical presentations may also include sudden infant death syndrome (SIDS; death of a child during the first year of life without an identifiable cause) and sudden unexpected nocturnal death syndrome (SUNDS), a typical presentation in individuals from Southeast Asia. Other conduction defects can include first-degree AV block, intraventricular conduction delay, right bundle branch block, and sick sinus syndrome. [from GeneReviews]
Ramon Brugada  |  Oscar Campuzano  |  Georgia Sarquella-Brugada, et. al.   view full author information

Additional description

From MedlinePlus Genetics
Brugada syndrome usually becomes apparent in adulthood, although it can develop any time throughout life. Signs and symptoms related to arrhythmias, including sudden death, can occur from early infancy to late adulthood. Sudden death typically occurs around age 40. This condition may explain some cases of sudden infant death syndrome (SIDS), which is a major cause of death in babies younger than 1 year. SIDS is characterized by sudden and unexplained death, usually during sleep.

Brugada syndrome is a condition that causes a disruption of the heart's normal rhythm. Specifically, this disorder can lead to irregular heartbeats in the heart's lower chambers (ventricles), which is an abnormality called ventricular arrhythmia. If untreated, the irregular heartbeats can cause fainting (syncope), seizures, difficulty breathing, or sudden death. These complications typically occur when an affected person is resting or asleep.

Sudden unexplained nocturnal death syndrome (SUNDS) is a condition characterized by unexpected cardiac arrest in young adults, usually at night during sleep. This condition was originally described in Southeast Asian populations, where it is a major cause of death. Researchers have determined that SUNDS and Brugada syndrome are the same disorder.  https://medlineplus.gov/genetics/condition/brugada-syndrome

Clinical features

From HPO
Sudden cardiac death
MedGen UID:
Concept ID:
Pathologic Function
The heart suddenly and unexpectedly stops beating resulting in death within a short time period (generally within 1 h of symptom onset).
Atrial fibrillation
MedGen UID:
Concept ID:
An atrial arrhythmia characterized by disorganized atrial activity without discrete P waves on the surface EKG, but instead by an undulating baseline or more sharply circumscribed atrial deflections of varying amplitude an frequency ranging from 350 to 600 per minute.
Atrial flutter
MedGen UID:
Concept ID:
Pathologic Function
A type of atrial arrhythmia characterized by atrial rates of between 240 and 400 beats per minute and some degree of atrioventricular node conduction block. Typically, the ventricular rate is half the atrial rate. In the EKG; atrial flutter waves are observed as sawtooth-like atrial activity. Pathophysiologically, atrial flutter is a form of atrial reentry in which there is a premature electrical impulse creates a self-propagating circuit.
Cardiac arrest
MedGen UID:
Concept ID:
An abrupt loss of heart function.
MedGen UID:
Concept ID:
Sign or Symptom
Syncope is a syndrome in which loss of consciousness is of relatively sudden onset, temporary (usually less than 1 to 2 minutes), self-terminating, and of usually rapid recovery. Syncope leads to a generalized weakness of muscles with loss of postural tone, inability to stand upright, and loss of consciousness. Once the patient is in a horizontal position, blood flow to the brain is no longer hindered by gravitation and consciousness is regained. Unconsciousness usually lasts for seconds to minutes. Headache and drowsiness (which usually follow seizures) do not follow a syncopal attack. Syncope results from a sudden impairment of brain metabolism usually due to a reduction in cerebral blood flow.
Ventricular fibrillation
MedGen UID:
Concept ID:
Disease or Syndrome
Uncontrolled contractions of muscles fibers in the left ventricle not producing contraction of the left ventricle. Ventricular fibrillation usually begins with a ventricular premature contraction and a short run of rapid ventricular tachycardia degenerating into uncoordinating ventricular fibrillations.
Right bundle branch block
MedGen UID:
Concept ID:
Disease or Syndrome
A conduction block of the right branch of the bundle of His. This manifests as a prolongation of the QRS complex (greater than 0.12 s) with delayed activation of the right ventricle and terminal delay on the EKG.
Supraventricular tachycardia with an accessory connection mediated pathway
MedGen UID:
Concept ID:
Disease or Syndrome
Supraventricular tachycardia in which an accessory pathway connecting the atria and ventricles, apart from the AV node, participates as a necessary part of a reentrant mechanism.

Professional guidelines


Bai R, Napolitano C, Bloise R, Monteforte N, Priori SG
Circ Arrhythm Electrophysiol 2009 Feb;2(1):6-15. Epub 2009 Feb 10 doi: 10.1161/CIRCEP.108.782888. PMID: 19808439

Recent clinical studies


Fan K, Lee K, Lau CP
J Interv Card Electrophysiol 1999 Oct;3(3):239-45. doi: 10.1023/a:1009847707872. PMID: 10490480


Abdelsayed M, Baruteau AE, Gibbs K, Sanatani S, Krahn AD, Probst V, Ruben PC
J Physiol 2017 Sep 15;595(18):6165-6186. Epub 2017 Aug 20 doi: 10.1113/JP274536. PMID: 28734073Free PMC Article
Sovari AA, Prasun MA, Kocheril AG
MedGenMed 2007 Sep 20;9(3):59. PMID: 18092065Free PMC Article
Tan HL, Hofman N, van Langen IM, van der Wal AC, Wilde AA
Circulation 2005 Jul 12;112(2):207-13. Epub 2005 Jul 5 doi: 10.1161/CIRCULATIONAHA.104.522581. PMID: 15998675


Stix G, Bella PD, Carbucicchio C, Schmidinger H
J Cardiovasc Electrophysiol 2000 May;11(5):516-21. doi: 10.1111/j.1540-8167.2000.tb00004.x. PMID: 10826930


Fan K, Lee K, Lau CP
J Interv Card Electrophysiol 1999 Oct;3(3):239-45. doi: 10.1023/a:1009847707872. PMID: 10490480

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