Brugada syndrome: Difference between revisions

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{{Use dmy dates|date=August 2018}}
{{SI}}  
{{good article}}
{{Infobox medical condition
{{Infobox medical condition (new)
| name            = Brugada syndrome
| name            = Brugada syndrome
| synonyms        = Sudden unexplained nocturnal death syndrome,<br> bangungut, pokkuri death syndrome
| image          = [[File:Brugada_syndrome_ECGs.jpg|left|thumb|ECG patterns in Brugada syndrome]]
| image          = Brugada EKG Schema.jpg
| caption        = ECG patterns in Brugada syndrome
|alt=| caption        = (A) Normal electrocardiogram of the precordial leads V<sub>1-3</sub>,<br>(B) changes in Brugada syndrome (type B)
| pronounce      =
| field          = [[Cardiology]]
| field          = [[Cardiology]]
| symptoms        = [[Syncope (medicine)|Passing out]], <br>[[sudden cardiac death]]
| symptoms        = [[Syncope]], [[sudden cardiac death]]
| complications  =
| onset          = Typically in [[adulthood]]
| onset          = Adulthood<ref name=Pol2017/>
| duration        = Lifelong
| duration        =  
| causes          = [[Genetic mutation]]s, primarily in the [[SCN5A]] gene
| types          =
| risks          = Family history, male gender, [[fever]]
| causes          = Genetics, certain medication<ref name=Pol2017/>
| risks          = Family history, [[Asian people|Asian]] descent, male
| diagnosis      = [[Electrocardiogram]] (ECG), [[genetic testing]]
| diagnosis      = [[Electrocardiogram]] (ECG), [[genetic testing]]
| differential    = [[Romano-Ward syndrome]], [[arrhythmogenic cardiomyopathy]],<br> [[Duchenne muscular dystrophy]]
| differential    = [[Long QT syndrome]], [[arrhythmogenic right ventricular dysplasia]]
| prevention      =  
| prevention      = Avoidance of certain medications, treatment of fever
| treatment      = [[Watchful waiting]], [[implantable cardioverter defibrillator]] (ICD)
| treatment      = [[Implantable cardioverter-defibrillator]] (ICD), medication
| medication     =
| prognosis      = Variable, risk of sudden death
| prognosis      =  
| frequency      = 1 in 5,000 to 10,000 people
| frequency      = 5 per 10,000
| deaths          = 8% of [[sudden cardiac death]]
}}
}}
'''Brugada syndrome''' (BrS) is a genetic condition characterized by abnormal electrical activity in the heart, leading to an increased risk of irregular heartbeats and sudden cardiac death. Individuals with Brugada syndrome may experience episodes of syncope (fainting), often triggered by rest or fever.
Brugada Syndrome
 
Brugada syndrome is a genetic disorder that results in abnormal electrical activity within the heart, leading to an increased risk of sudden cardiac death. It is characterized by a specific pattern on an electrocardiogram (ECG) and is often associated with [[ventricular fibrillation]].
== Overview ==
==Epidemiology==
 
Brugada syndrome is more prevalent in certain populations, particularly in Southeast Asia. It is estimated to affect 1 in 2,000 people worldwide. The condition is more common in males than females, with a male-to-female ratio of approximately 8:1.
Brugada syndrome primarily affects the heart's rhythm, causing disturbances in the heart's electrical signals. These abnormalities can lead to life-threatening arrhythmias, including ventricular fibrillation and ventricular tachycardia, which can cause cardiac arrest and sudden death if not treated immediately.
==Genetics==
 
Brugada syndrome is primarily inherited in an [[autosomal dominant]] manner. Mutations in the [[SCN5A]] gene, which encodes the cardiac sodium channel, are the most common genetic cause. However, mutations in other genes such as [[CACNA1C]], [[CACNB2]], and [[SCN1B]] have also been implicated.
== Signs and Symptoms ==
==Pathophysiology==
 
The hallmark of Brugada syndrome is the disruption of the normal electrical activity of the heart, particularly in the right ventricular outflow tract. This disruption is often due to a loss of function in the sodium channels, leading to a predisposition to [[ventricular arrhythmias]].
Individuals with Brugada syndrome may experience the following symptoms:
==Clinical Presentation==
 
Patients with Brugada syndrome may be asymptomatic or present with symptoms such as:
Syncope (fainting) episodes, often during sleep or rest
* [[Syncope]]
Palpitations
* Palpitations
Seizures
* Sudden cardiac arrest
Difficulty breathing
The condition is often diagnosed after a family member experiences sudden cardiac death or during an evaluation for syncope.
Abnormal heart rhythms, typically revealed in an electrocardiogram (ECG)
==Diagnosis==
In some cases, the first indication of Brugada syndrome may be sudden cardiac arrest or death.
The diagnosis of Brugada syndrome is primarily based on the characteristic ECG findings, which include:
 
* Coved-type ST-segment elevation in the right precordial leads (V1-V3)
== Causes ==
A [[provocative test]] with sodium channel blockers such as [[ajmaline]] or [[flecainide]] may be used to unmask the ECG pattern in suspected cases.
 
==Management==
Brugada syndrome is a genetic condition, typically inherited in an autosomal dominant manner. Most cases are associated with mutations in the SCN5A gene, which codes for a sodium ion channel in the heart cells crucial for maintaining normal heart rhythm.
The management of Brugada syndrome focuses on preventing sudden cardiac death. Treatment options include:
 
* [[Implantable cardioverter-defibrillator]] (ICD) for high-risk patients
== Diagnosis ==
* Avoidance of drugs that can exacerbate the condition
 
* Lifestyle modifications, such as avoiding excessive alcohol and fever management
Diagnosis of Brugada syndrome typically involves an ECG, which can detect characteristic patterns indicative of the syndrome. The condition can be further confirmed with genetic testing. Provocative drug testing using sodium channel blockers can also aid in diagnosis.
==Prognosis==
 
The prognosis of Brugada syndrome varies depending on the presence of symptoms and the risk of arrhythmias. Asymptomatic individuals with a normal ECG have a lower risk of sudden cardiac death compared to those with a history of syncope or documented ventricular arrhythmias.
== Treatment ==
==Gallery==
 
<gallery>
The primary treatment strategy for Brugada syndrome focuses on the prevention of sudden cardiac death. Implantable cardioverter defibrillators (ICDs) are often used in high-risk individuals. Medications such as quinidine can also be used to reduce the risk of arrhythmias. Additionally, lifestyle modifications and avoidance of triggers such as fever and certain medications are recommended.
File:Brugada syndrome ECGs.jpg|Brugada syndrome ECGs
 
File:Wavebreak and re-entry as a mechanism of arrhythmias.png|Wavebreak and re-entry as a mechanism of arrhythmias
== Prognosis ==
File:Brugada.jpg|Brugada
 
File:Brugada syndrome type1 example1 (CardioNetworks ECGpedia).png|Brugada syndrome type 1 example 1
The prognosis for individuals with Brugada syndrome varies widely, depending on the severity of the condition and the individual's response to treatment. With appropriate management, individuals with Brugada syndrome can lead a normal life.
File:Brugada syndrome type2 example1 (CardioNetworks ECGpedia).png|Brugada syndrome type 2 example 1
 
File:AICD.jpg|AICD
== Epidemiology ==
</gallery>
 
==See Also==
Brugada syndrome affects both males and females, though it appears more commonly in males. It has a global distribution, with a higher prevalence reported in Southeast Asia.
* [[Long QT syndrome]]
[[Category:Cardiovascular diseases]]
* [[Catecholaminergic polymorphic ventricular tachycardia]]
[[Category:Genetic disorders]]
==External Links==
{{stub}}
* [Brugada Syndrome Foundation](https://www.brugada.org)
{{Channelopathy}}
{{Cardiology}}
{{DEFAULTSORT:Brugada Syndrome}}
{{Genetic disorders}}
[[Category:Cardiac arrhythmia]]
[[Category:Cardiac electrophysiology]]
[[Category:Channelopathies]]
[[Category:Genetic disorders with OMIM but no gene]]
[[Category:Congenital disorders]]
[[Category:Rare diseases]]
[[Category:Syndromes affecting the heart]]
[[Category:Cardiogenetic disorders]]
[[Category:RTT]]
[[Category:Rare syndromes]]

Latest revision as of 19:30, 4 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Brugada syndrome
ECG patterns in Brugada syndrome
Synonyms N/A
Pronounce N/A
Specialty N/A
Symptoms Syncope, sudden cardiac death
Complications N/A
Onset Typically in adulthood
Duration Lifelong
Types N/A
Causes Genetic mutations, primarily in the SCN5A gene
Risks Family history, male gender, fever
Diagnosis Electrocardiogram (ECG), genetic testing
Differential diagnosis Long QT syndrome, arrhythmogenic right ventricular dysplasia
Prevention Avoidance of certain medications, treatment of fever
Treatment Implantable cardioverter-defibrillator (ICD), medication
Medication N/A
Prognosis Variable, risk of sudden death
Frequency 1 in 5,000 to 10,000 people
Deaths N/A


Brugada Syndrome Brugada syndrome is a genetic disorder that results in abnormal electrical activity within the heart, leading to an increased risk of sudden cardiac death. It is characterized by a specific pattern on an electrocardiogram (ECG) and is often associated with ventricular fibrillation.

Epidemiology[edit]

Brugada syndrome is more prevalent in certain populations, particularly in Southeast Asia. It is estimated to affect 1 in 2,000 people worldwide. The condition is more common in males than females, with a male-to-female ratio of approximately 8:1.

Genetics[edit]

Brugada syndrome is primarily inherited in an autosomal dominant manner. Mutations in the SCN5A gene, which encodes the cardiac sodium channel, are the most common genetic cause. However, mutations in other genes such as CACNA1C, CACNB2, and SCN1B have also been implicated.

Pathophysiology[edit]

The hallmark of Brugada syndrome is the disruption of the normal electrical activity of the heart, particularly in the right ventricular outflow tract. This disruption is often due to a loss of function in the sodium channels, leading to a predisposition to ventricular arrhythmias.

Clinical Presentation[edit]

Patients with Brugada syndrome may be asymptomatic or present with symptoms such as:

  • Syncope
  • Palpitations
  • Sudden cardiac arrest

The condition is often diagnosed after a family member experiences sudden cardiac death or during an evaluation for syncope.

Diagnosis[edit]

The diagnosis of Brugada syndrome is primarily based on the characteristic ECG findings, which include:

  • Coved-type ST-segment elevation in the right precordial leads (V1-V3)

A provocative test with sodium channel blockers such as ajmaline or flecainide may be used to unmask the ECG pattern in suspected cases.

Management[edit]

The management of Brugada syndrome focuses on preventing sudden cardiac death. Treatment options include:

  • Implantable cardioverter-defibrillator (ICD) for high-risk patients
  • Avoidance of drugs that can exacerbate the condition
  • Lifestyle modifications, such as avoiding excessive alcohol and fever management

Prognosis[edit]

The prognosis of Brugada syndrome varies depending on the presence of symptoms and the risk of arrhythmias. Asymptomatic individuals with a normal ECG have a lower risk of sudden cardiac death compared to those with a history of syncope or documented ventricular arrhythmias.

Gallery[edit]

  • Brugada syndrome ECGs
    Brugada syndrome ECGs
  • Wavebreak and re-entry as a mechanism of arrhythmias
    Wavebreak and re-entry as a mechanism of arrhythmias
  • Brugada
    Brugada
  • Brugada syndrome type 1 example 1
    Brugada syndrome type 1 example 1
  • Brugada syndrome type 2 example 1
    Brugada syndrome type 2 example 1
  • AICD
    AICD

See Also[edit]

External Links[edit]



Cardiovascular disease A-Z

Most common cardiac diseases

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Genetic disorders relating to deficiencies of transcription factor or coregulators
(1) Basic domains
(2) Zinc finger
DNA-binding domains
(3) Helix-turn-helix domains
3.1
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3.5
(4) β-Scaffold factors
with minor groove contacts
(0) Other transcription factors
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