Brugada syndrome
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Brugada syndrome
Cardiovascular system
Cardiac arrhythmias
Atrioventricular block
Bundle branch block
Pulseless electrical activity
Atrial fibrillation
Atrial flutter
Atrioventricular nodal reentrant tachycardia (AVNRT)
Premature atrial contraction
Wolff-Parkinson-White syndrome
Brugada syndrome
Long QT syndrome and Torsade de pointes
Premature ventricular contraction
Ventricular fibrillation
Ventricular tachycardia
Cardiac tumors
Cardiac tumors
Shock
Shock
Vascular disorders
Arterial disease
Aneurysms
Aortic dissection
Angina pectoris
Coronary steal syndrome
Myocardial infarction
Prinzmetal angina
Stable angina
Unstable angina
Abetalipoproteinemia
Familial hypercholesterolemia
Hyperlipidemia
Hypertriglyceridemia
Coarctation of the aorta
Conn syndrome
Cushing syndrome
Hypertension
Hypertensive emergency
Pheochromocytoma
Polycystic kidney disease
Renal artery stenosis
Hypotension
Orthostatic hypotension
Lymphangioma
Lymphedema
Peripheral artery disease
Subclavian steal syndrome
Nutcracker syndrome
Superior mesenteric artery syndrome
Angiosarcomas
Human herpesvirus 8 (Kaposi sarcoma)
Vascular tumors
Behcet's disease
Kawasaki disease
Vasculitis
Chronic venous insufficiency
Deep vein thrombosis
Thrombophlebitis
Cardiovascular system pathology review
Acyanotic congenital heart defects: Pathology review
Aortic dissections and aneurysms: Pathology review
Atherosclerosis and arteriosclerosis: Pathology review
Cardiac and vascular tumors: Pathology review
Cardiomyopathies: Pathology review
Coronary artery disease: Pathology review
Cyanotic congenital heart defects: Pathology review
Dyslipidemias: Pathology review
Endocarditis: Pathology review
Heart blocks: Pathology review
Heart failure: Pathology review
Hypertension: Pathology review
Pericardial disease: Pathology review
Peripheral artery disease: Pathology review
Shock: Pathology review
Supraventricular arrhythmias: Pathology review
Valvular heart disease: Pathology review
Vasculitis: Pathology review
Ventricular arrhythmias: Pathology review
Assessments
Brugada syndrome
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Brugada syndrome
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If left untreated this patient is at risk for which of the following complications?
External References
First Aid
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2016
Autosomal dominant disorders
Brugada syndrome p. 301
Brugada syndrome p. 314, 310
Transcript
Content Reviewers
Brugada syndrome is named after two Spanish brothers, Pedro and Josep Brugada, who recognized a specific pattern of ventricular fibrillation on the electrocardiogram or ECG, of previously healthy individuals who had a sudden death! They identified this as Brugada syndrome and found that some cases could be traced back to a specific genetic cause.
Many individuals with Brugada syndrome don’t have an identifiable genetic cause, but in some individuals, there is one. The most well-known cause is a mutation in the gene SCN5A. The SCN5A mutation is inherited in an autosomal dominant pattern, meaning a single mutation is enough to cause the disease.
The gene codes for sodium ion channels in the cell membranes of cardiac muscle cells, and a faulty sodium ion channel affects the heart cell’s ability to conduct an action potential. So the mutation results in some regions of the heart having abnormal repolarization. In some cases, the heart might have a normal rhythm but then develop into a Brugada syndrome in the presence of certain medications like sodium channel blockers.
On an electrocardiogram, Brugada syndrome typically has ST elevations (which are often a sign of ventricular strain), as well as a right bundle branch block, which indicates that the ventricles aren’t depolarizing normally. As a result, these regions become susceptible to a reentrant loop, which is when a depolarization signal starts going around and around in a loop, causing ventricular tachycardia and sometimes ventricular fibrillation.
Ventricular fibrillation, sometimes called v-fib, means the heart’s muscle fibers start quivering because they’re not contracting at the same time. Normally, an electrical signal spreads fast enough so that all of the muscle fibers in the ventricles contract almost at the same time, which essentially looks like a single, coordinated contraction.
Summary
Brugada syndrome is a potentially life-threatening condition that can cause sudden cardiac death. The condition is characterized by abnormal heart rhythms (arrhythmias) on ECG and a predisposition to developing ventricular fibrillation, a particularly dangerous type of arrhythmia. People with Brugada syndrome often have no symptoms, making the condition difficult to diagnose. However, in some cases, people with the condition may experience episodes of palpitations, fatigue, and shortness of breath.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "Brugada syndrome: Diagnosis, risk stratification and management" Archives of Cardiovascular Diseases (2017)
- "Brugada Syndrome" Methodist DeBakey Cardiovascular Journal (2014)
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