Brugada syndrome: Clinical presentation, diagnosis, and evaluation
- John V Wylie, MD, FACC
John V Wylie, MD, FACC
- Assistant Professor of Medicine
- Tufts Medical School
- Ann C Garlitski, MD, FACC, FHRS
Ann C Garlitski, MD, FACC, FHRS
- Assistant Professor of Medicine
- Hofstra North Shore-LIJ School of Medicine
- Hofstra University
- Section Editors
- Scott Manaker, MD, PhD
Scott Manaker, MD, PhD
- Section Editor — Critical Care
- Professor of Medicine
- University of Pennsylvania School of Medicine
- Samuel Asirvatham, MD
Samuel Asirvatham, MD
- Section Editor — Long QT Syndrome
- Professor of Medicine and Pediatrics
- Mayo Clinic College of Medicine
The vast majority of cases of sudden cardiac arrest (SCA) and sudden cardiac death (SCD) are caused by ventricular tachyarrhythmias, with most of these associated with structural heart disease, particularly coronary heart disease. SCA in the apparently normal heart is an uncommon occurrence, accounting for only 5 to 10 percent of SCA cases. (See "Pathophysiology and etiology of sudden cardiac arrest".)
Some causes of SCA in patients with apparently normal hearts have been identified and include:
●Congenital long QT syndrome (LQTS) (see "Clinical features of congenital long QT syndrome")
●Acquired LQTS with polymorphic ventricular tachycardia (VT) (see "Acquired long QT syndrome")
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- CLINICAL PRESENTATION
- Definition of Brugada pattern versus Brugada syndrome
- Sudden cardiac arrest and syncope
- Atrial fibrillation
- Nocturnal agonal respiration
- Sudden unexpected nocturnal death syndrome
- Features in children
- ECG findings
- - Type 1 versus Type 2
- - Variation of ECG findings over time
- - Provoking factors
- Medications and toxins
- 2013 HRS/EHRA/APHRS criteria
- 2005 HRS/EHRA criteria
- EVALUATION AND RISK STRATIFICATION
- Our approach to evaluation and risk stratification
- Testing for underlying heart disease
- Drug challenge
- - Drug challenge procedure
- Signal-averaged ECG
- 12-lead ECG
- Electrophysiology testing
- Genetic testing
- DIFFERENTIAL DIAGNOSIS
- Differential diagnosis of Brugada pattern ECG findings
- - Brugada ECG pattern in ARVC
- Differential diagnosis for VT or sudden death with a structurally normal heart
- SUMMARY AND RECOMMENDATIONS