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Brugada Syndrome
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Contents
Other Names
Background
Brugada syndrome is a genetic electrical disorder of the heart. There are 3 types of Brugada syndrome with 20 types of genetic defects. The most common type is a sodium channel defect. It can lead to syncope and also sudden death. There is no known cure but it can be treated medically and also with an ICD for high risk patient.
Pathophysiology
- Autosomal dominant inheritance [1]
- A loss of function mutation of the SCN5A gene is implicated in 20% of Brugada syndrome patients [2]
Clinical Features
- Patients are at risk for polymorphic ventricular tachyarrhythmias
Differential Diagnosis
Risk Factors
Evaluation
- An ECG is used to evaluate these patients, looking for 3 potential patterns.
- Genetic testing for BrS is recommended in patients who have family members with an identified causative mutation for Brugada syndrome, patients with Brugada type I when a Cardiologist has suspicion for BrS based on history or physical exam and in patients who have persistent or induced with provocative testing type I Brugada ECG findings
Classification
- Type I Brugada syndrome is defined as ST-segment elevation greater than or equal to 2mm in more than 1 lead among the right precordial leads (V1 and V2)
Management
Complications
See Also
References
- ↑ Silvia Priori, A. W. (2013). Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. European Society of Cardiology, 1389-1406.
- ↑ Ackerman, M. (2004). Cardiac channelopathies: it’s in the genes. Nature Medicine, 463-464.
Created by:
John Kiel on 13 June 2019 09:12:25
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Last edited:
27 August 2019 11:36:55
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