Brugada syndrome: Difference between revisions
From WikiMD's Wellness Encyclopedia
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{{SI}} | |||
{{Infobox medical condition | |||
| name = Brugada syndrome | |||
| image = [[File:Brugada_syndrome_ECGs.jpg|left|thumb|ECG patterns in Brugada syndrome]] | |||
| caption = ECG patterns in Brugada syndrome | |||
| field = [[Cardiology]] | |||
| symptoms = [[Syncope]], [[sudden cardiac death]] | |||
| onset = Typically in [[adulthood]] | |||
| duration = Lifelong | |||
| causes = [[Genetic mutation]]s, primarily in the [[SCN5A]] gene | |||
| risks = Family history, male gender, [[fever]] | |||
| diagnosis = [[Electrocardiogram]] (ECG), [[genetic testing]] | |||
| differential = [[Long QT syndrome]], [[arrhythmogenic right ventricular dysplasia]] | |||
| prevention = Avoidance of certain medications, treatment of fever | |||
| treatment = [[Implantable cardioverter-defibrillator]] (ICD), medication | |||
| prognosis = Variable, risk of sudden death | |||
| frequency = 1 in 5,000 to 10,000 people | |||
}} | |||
Brugada Syndrome | 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]]. | 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== | ==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 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== | ==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. | 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== | ==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]]. | 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== | ==Clinical Presentation== | ||
Patients with Brugada syndrome may be asymptomatic or present with symptoms such as: | Patients with Brugada syndrome may be asymptomatic or present with symptoms such as: | ||
| Line 17: | Line 30: | ||
* Palpitations | * Palpitations | ||
* Sudden cardiac arrest | * Sudden cardiac arrest | ||
The condition is often diagnosed after a family member experiences sudden cardiac death or during an evaluation for syncope. | The condition is often diagnosed after a family member experiences sudden cardiac death or during an evaluation for syncope. | ||
==Diagnosis== | ==Diagnosis== | ||
The diagnosis of Brugada syndrome is primarily based on the characteristic ECG findings, which include: | 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) | * 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. | A [[provocative test]] with sodium channel blockers such as [[ajmaline]] or [[flecainide]] may be used to unmask the ECG pattern in suspected cases. | ||
==Management== | ==Management== | ||
The management of Brugada syndrome focuses on preventing sudden cardiac death. Treatment options include: | The management of Brugada syndrome focuses on preventing sudden cardiac death. Treatment options include: | ||
| Line 31: | Line 40: | ||
* Avoidance of drugs that can exacerbate the condition | * Avoidance of drugs that can exacerbate the condition | ||
* Lifestyle modifications, such as avoiding excessive alcohol and fever management | * Lifestyle modifications, such as avoiding excessive alcohol and fever management | ||
==Prognosis== | ==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 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== | |||
<gallery> | |||
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 | |||
File:Brugada.jpg|Brugada | |||
File:Brugada syndrome type1 example1 (CardioNetworks ECGpedia).png|Brugada syndrome type 1 example 1 | |||
File:Brugada syndrome type2 example1 (CardioNetworks ECGpedia).png|Brugada syndrome type 2 example 1 | |||
File:AICD.jpg|AICD | |||
</gallery> | |||
==See Also== | ==See Also== | ||
* [[Long QT syndrome]] | * [[Long QT syndrome]] | ||
* [[Catecholaminergic polymorphic ventricular tachycardia]] | * [[Catecholaminergic polymorphic ventricular tachycardia]] | ||
==External Links== | ==External Links== | ||
* [Brugada Syndrome Foundation](https://www.brugada.org) | * [Brugada Syndrome Foundation](https://www.brugada.org) | ||
{{Cardiology}} | {{Cardiology}} | ||
{{Genetic disorders}} | {{Genetic disorders}} | ||
| Line 47: | Line 61: | ||
[[Category:Genetic disorders with OMIM but no gene]] | [[Category:Genetic disorders with OMIM but no gene]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
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 | |
|---|---|
 | |
| 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 -
Wavebreak and re-entry as a mechanism of arrhythmias -
Brugada -
Brugada syndrome type 1 example 1 -
Brugada syndrome type 2 example 1 -
AICD
.png)
.png)
See Also[edit]
External Links[edit]
- [Brugada Syndrome Foundation](https://www.brugada.org)
Cardiovascular disease A-Z
Most common cardiac diseases
- Cardiac arrhythmia
- Cardiogenetic disorders
- Cardiomegaly
- Cardiomyopathy
- Cardiopulmonary resuscitation
- Chronic rheumatic heart diseases
- Congenital heart defects
- Heart neoplasia
- Ischemic heart diseases
- Pericardial disorders
- Syndromes affecting the heart
- Valvular heart disease
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[edit]
- Accelerated idioventricular rhythm
- Acute decompensated heart failure
- Arteriosclerotic heart disease
- Athletic heart syndrome
- Atrial flutter
- Atrioventricular fistula
- Cardiovascular disease in Australia
- Autoimmune heart disease
B[edit]
C[edit]
- Ebb Cade
- Cardiac allograft vasculopathy
- Cardiac amyloidosis
- Cardiac asthma
- Cardiac tamponade
- Cardiogenic shock
- Cardiogeriatrics
- Cardiorenal syndrome
- Cardiotoxicity
- Carditis
- Coronary artery aneurysm
- Coronary artery anomaly
- Coronary artery disease
- Spontaneous coronary artery dissection
- Coronary artery ectasia
- Coronary occlusion
- Coronary steal
- Coronary thrombosis
- Coronary vasospasm
- Cœur en sabot
- Coxsackievirus-induced cardiomyopathy
D[edit]
E[edit]
H[edit]
- Heart attack
- Heart failure
- Heart failure with preserved ejection fraction
- Heart to Heart (1949 film)
- High-output heart failure
- Hyperdynamic precordium
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[edit]
- Idiopathic giant-cell myocarditis
- Interventricular dyssynchrony
- Intraventricular dyssynchrony
- Isolated atrial amyloidosis
K[edit]
L[edit]
M[edit]
- Mydicar
- Myocardial bridge
- Myocardial disarray
- Myocardial rupture
- Myocardial scarring
- Myocardial stunning
- Myocarditis
N[edit]
O[edit]
P[edit]
- Papillary fibroelastoma
- Pathophysiology of heart failure
- Postpericardiotomy syndrome
- Pulmonary vein stenosis
R[edit]
S[edit]
- Saturated fat and cardiovascular disease
- SCAR-Fc
- Shone's syndrome
- Strain pattern
- Subacute bacterial endocarditis
- Sudden cardiac death of athletes
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[edit]
V[edit]
W[edit]
| Genetic disorders relating to deficiencies of transcription factor or coregulators | ||||||||||||||||||||||||||||||||||
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