Steven J Fowler1, Silvia G Priori. 1. Clinical Cardiac Electrophysiology, Department of Cardiology, New York University Langone Medical Center, New York, USA.
Abstract
PURPOSE OF REVIEW: The Brugada syndrome continues to spark intensive investigation since its earliest description. New insight has been gained regarding the genetic, histopathologic, and metabolic mechanisms of this heritable channelopathy - a heterogeneity that is reflected in the diverse clinical presentation. In this review, we will focus on clinical spectrum of patients with a Brugada ECG pattern with a special focus on diagnosis and risk stratification. RECENT FINDINGS: In the past year, multiple case reports have demonstrated that the Brugada ECG pattern is not limited to those with the diagnosed syndrome, with increasing evidence that an 'overlap' exists among inherited channelopathies, especially those involving the sodium and calcium channels. SUMMARY: The clinical spectrum of patients with a Brugada ECG remains broad, reflecting the heterogeneity of the underlying pathophysiology. As the true prevalence of the disease unfolds, knowledge regarding the clinical spectrum of patients with a Brugada ECG is important to implement effective risk stratification and management. It may be proposed that the ECG pattern of ST-segment elevation in the right precordial leads should not be seen as a marker of a specific syndrome, but rather as a common electrical expression of structural abnormalities in the right ventricle that may have genetic, infective, or inflammatory origins.
PURPOSE OF REVIEW: The Brugada syndrome continues to spark intensive investigation since its earliest description. New insight has been gained regarding the genetic, histopathologic, and metabolic mechanisms of this heritable channelopathy - a heterogeneity that is reflected in the diverse clinical presentation. In this review, we will focus on clinical spectrum of patients with a Brugada ECG pattern with a special focus on diagnosis and risk stratification. RECENT FINDINGS: In the past year, multiple case reports have demonstrated that the Brugada ECG pattern is not limited to those with the diagnosed syndrome, with increasing evidence that an 'overlap' exists among inherited channelopathies, especially those involving the sodium and calcium channels. SUMMARY: The clinical spectrum of patients with a Brugada ECG remains broad, reflecting the heterogeneity of the underlying pathophysiology. As the true prevalence of the disease unfolds, knowledge regarding the clinical spectrum of patients with a Brugada ECG is important to implement effective risk stratification and management. It may be proposed that the ECG pattern of ST-segment elevation in the right precordial leads should not be seen as a marker of a specific syndrome, but rather as a common electrical expression of structural abnormalities in the right ventricle that may have genetic, infective, or inflammatory origins.
Authors: Paula G Macedo; Josep Brugada; Pavel Leinveber; Begoña Benito; Irma Molina; Fatima Sert-Kuniyoshi; Taro Adachi; Jan Bukartyk; Christelle van der Walt; Tomas Konecny; Shantal Maharaj; Tomas Kara; Josep Montserrat; Virend Somers Journal: Am J Cardiol Date: 2011-01-19 Impact factor: 2.778