Brugada syndrome: Difference between revisions

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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

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

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

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

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

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

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

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.

External Links

[Brugada Syndrome Foundation](https://www.brugada.org)

Cardiovascular disease A-Z

Most common cardiac diseases

A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z

A

B

Brown atrophy of the heart

C

D

E

H

A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z

I

K

Keshan disease

L

Loeffler endocarditis

M

N

Nonbacterial thrombotic endocarditis

O

P

R

S

A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z

T

Traumatic cardiac arrest

V

W

Waon therapy

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-{{Use dmy dates|date=August 2018}}
+Brugada Syndrome
-{{good article}}
-{{Infobox medical condition (new)
-| name            = Brugada syndrome
-| synonyms        = Sudden unexplained nocturnal death syndrome,<br> bangungut, pokkuri death syndrome
-| image           = Brugada EKG Schema.jpg
-|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]]
-| symptoms        = [[Syncope (medicine)|Passing out]], <br>[[sudden cardiac death]]
-| complications   =
-| onset           = Adulthood<ref name=Pol2017/>
-| duration        =
-| types           =
-| causes          = Genetics, certain medication<ref name=Pol2017/>
-| risks           = Family history, [[Asian people|Asian]] descent, male
-| diagnosis       = [[Electrocardiogram]] (ECG), [[genetic testing]]
-| differential    = [[Romano-Ward syndrome]], [[arrhythmogenic cardiomyopathy]],<br> [[Duchenne muscular dystrophy]]
-| prevention      =
-| treatment       = [[Watchful waiting]], [[implantable cardioverter defibrillator]] (ICD)
-| medication      =
-| prognosis       =
-| 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.
-== Overview ==
+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]].
-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.
+==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.
-== Signs and Symptoms ==
+==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.
-Individuals with Brugada syndrome may experience the following 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]].
-Syncope (fainting) episodes, often during sleep or rest
+==Clinical Presentation==
-Palpitations
+Patients with Brugada syndrome may be asymptomatic or present with symptoms such as:
-Seizures
+* [[Syncope]]
-Difficulty breathing
+* Palpitations
-Abnormal heart rhythms, typically revealed in an electrocardiogram (ECG)
+* Sudden cardiac arrest
-In some cases, the first indication of Brugada syndrome may be sudden cardiac arrest or death.
-== Causes ==
+The condition is often diagnosed after a family member experiences sudden cardiac death or during an evaluation for syncope.
-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.
+==Diagnosis==
+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)
-== Diagnosis ==
+A [[provocative test]] with sodium channel blockers such as [[ajmaline]] or [[flecainide]] may be used to unmask the ECG pattern in suspected cases.
-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.
+==Management==
+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
-== Treatment ==
+==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.
-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.
+==See Also==
+* [[Long QT syndrome]]
+* [[Catecholaminergic polymorphic ventricular tachycardia]]
-== Prognosis ==
+==External Links==
+* [Brugada Syndrome Foundation](https://www.brugada.org)
-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.
+{{Cardiology}}
+{{Genetic disorders}}
-== Epidemiology ==
+[[Category:Cardiac electrophysiology]]
+[[Category:Genetic disorders with OMIM but no gene]]
-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.
+[[Category:Rare diseases]]
-[[Category:Cardiovascular diseases]]
-[[Category:Genetic disorders]]
-{{stub}}
-{{Channelopathy}}
-{{DEFAULTSORT:Brugada Syndrome}}
-[[Category:Cardiac arrhythmia]]
-[[Category:Channelopathies]]
-[[Category:Congenital disorders]]
-[[Category:Syndromes affecting the heart]]
-[[Category:Cardiogenetic disorders]]
-[[Category:RTT]]
-[[Category:Rare syndromes]]