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Update on the Differential Diagnosis and Treatment of Brugada Syndrome

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J Wave Syndromes

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Abstract

Brugada Syndrome (BrS) is a channelopathy that predisposes to sudden death (SD) in the absence of structural heart disease. The diagnostic pattern, known as coved-type or type 1, consists of a concave ST segment elevation ≥2 mm followed by a negative T wave, and it must be distinguished from other conditions that also present with ST-segment elevation in the precordial leads, including Brugada phenocopies (BrS type 1 pattern triggered by an environmental cause such as RV ischemia, acute pulmonary embolism, etc.) and other causes of Brugada-like ECG patterns, as acute ischemia of the left anterior descendent artery, right bundle branch block, early repolarization, arrhythmogenic right ventricular dysplasia, etc. Other modulating factors, such as fever, hormones or certain drugs, can unmask or exacerbate the typical BrS pattern.

Because the clinical phenotype can be widely variable, risk stratification is mandatory in all diagnosed patients. ICD is the only effective therapy to prevent SD. In case of arrhythmic storms or multiple shocks, quinidine and/or isoproterenol may be useful. Ablation of the epicardial substrate at the level of the right ventricular outflow tract has recently been proposed and can emerge as a real therapeutic alternative. In all patients, it is mandatory to avoid known triggers as fever and certain drugs. Genetic testing, although only positive in a minority of patients, can be useful to confirm borderline diagnosis and can be extremely valuable for family screening.

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Authors and Affiliations

  1. Arrhythmia Unit, Department of Cardiology, Thorax Institute, Hospital Clinic, Barcelona, Spain

    Elena Arbelo MD, PhD

  2. Cardiology Department, Hospital Clinic Barcelona, Barcelona, Spain

    Josep Brugada Terradellas MD, PhD

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  1. Elena Arbelo MD, PhD
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  2. Josep Brugada Terradellas MD, PhD
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Correspondence to Josep Brugada Terradellas MD, PhD .

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Editors and Affiliations

  1. Cardiovascular Research, Lankenau Institute for Medical Research, Wynnewood, Pennsylvania, USA

    Charles Antzelevitch

  2. Lankenau Institute for Medical Research, Lankenau Medical Center, Wynnewood, Pennsylvania, USA

    Gan-Xin Yan

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Arbelo, E., Brugada Terradellas, J. (2016). Update on the Differential Diagnosis and Treatment of Brugada Syndrome. In: Antzelevitch, C., Yan, GX. (eds) J Wave Syndromes. Springer, Cham. https://doi.org/10.1007/978-3-319-31578-2_7

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