Brugada syndrome and “Brugada sign”: Clinical spectrum with a guide for the clinician

• Published in: The American heart journal Vol. 145; no. 5; pp. 768 - 778
• Main Authors: Littmann, Laszlo, Monroe, Michael H, Kerns, William P, Svenson, Robert H, Gallagher, John J
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.05.2003; Elsevier; Elsevier Limited
• Subjects: Algorithms; Arrhythmias, Cardiac - diagnosis; Arrhythmias, Cardiac - physiopathology; Arrhythmias, Cardiac - therapy; Biological and medical sciences; Bundle-Branch Block - diagnosis; Bundle-Branch Block - physiopathology; Bundle-Branch Block - therapy; Cardiac arrhythmia; Cardiac dysrhythmias; Cardiology. Vascular system; Death, Sudden, Cardiac - prevention & control; Diagnosis, Differential; Electrocardiography; Electrocardiography - drug effects; Ethnicity; Family Health; Heart; Heart attacks; Humans; Medical sciences; Mutation; Patients; Prognosis; Syndrome; Water-Electrolyte Imbalance - physiopathology; Human; Symptomatology; Arrhythmia; Brugada syndrome; Heart disease; Cardiovascular disease; Diagnosis
• ISSN: 0002-8703; 1097-6744
• DOI: 10.1016/S0002-8703(03)00081-4

Patients with the manifest Brugada syndrome have an inordinate risk of sudden death and are candidates for implantation of a defibrillator. The Brugada type electrocardiogram (ECG) abnormality (the “Brugada sign”), however, is known to be associated with a wide range of conditions, many of which may not pose such a threat. Clinicians need guidance in choosing a rational approach for the evaluation and treatment of patients with a finding of the Brugada sign. A systematic literature search was performed to identify publications on the Brugada syndrome and the Brugada-type ECG abnormality, with special emphasis on analyzing outcomes data. In addition, the ECG database of our institution was reviewed for tracings consistent with the Brugada sign, and, when possible, clinical correlations were made. Patients with the Brugada sign and a family history of sudden death or a personal history of syncope are at a high risk of sudden death and therefore should be strongly considered for implantation of a defibrillator. In patients who are hospitalized and critically ill, the Brugada sign is frequently the result of severe hyperkalemia, drug toxicity, or right ventricular injury. In most individuals with no symptoms and without a family history of sudden death, the Brugada sign is likely a normal variant. Most patients with the Brugada sign can be risk-stratified with simple clinical tools. Specific testing for the Brugada syndrome should be reserved for questionable cases and for the research setting. A provisional diagnostic-therapeutic algorithm is offered as a means of assisting the clinician in the evaluation and treatment of patients with the Brugada sign.