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Acquired Form of Brugada Syndrome

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Electrical Diseases of the Heart

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Abstract

Brugada syndrome is characterized by Type 1 (coved type) ST-segment elevation in the right ­precordial leads (V1–V3) and an episode of ventricular fibrillation (VF) in the absence of structural heart disease. Seven genotypes have been identified responsible for Brugada syndrome in approximately one-thirds of Brugada patients, and decreases in inward sodium or calcium current (fast sodium current [INa], L-type calcium current [ICa-L]) or increase in a transient outward potassium current (Ito) are considered to produce Brugada phenotype in the seven genotypes. On the other hand, experimental studies have suggested that an intrinsically prominent Ito-mediated action potential (AP) notch and a subsequent loss of AP dome in the epicardium, but not in the endocardium of the right ventricular outflow tract, give rise to a transmural voltage gradient, resulting in ST-segment elevation and phase 2 reentry-induced VF. Therefore, any drugs and interventions that increase outward currents (e.g. Ito, adenosine tri-phosphate sensitive potassium current [IK-ATP], slow and fast activating components of IK [Iks, IKr]) or decrease inward currents (e.g. fast INa, ICa-L) at the end of phase 1 of the AP can accentuate or unmask ST-segment elevation, similar to that found in Brugada syndrome, thus producing acquired forms of the Brugada syndrome. In this chapter, such drugs and conditions, which provoke transient Brugada phenotype, developing acquired forms of the Brugada syndrome, will be discussed.

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Acknowledgements.

Dr. W Shimizu was supported in part by the Research Grant for the Cardiovascular Diseases (H24-033) from the Ministry of Health, Labour and Welfare, Japan, and Grant-in-Aid for Scientific Research on Innovative Areas (22136011).

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  1. Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan

    Wataru Shimizu MD, PhD

  2. Division of Cardiology, Department of Internal Medicine, Nippon Medical School, 1-1-5 Sendagi Bunkyo-ku, Tokyo, 113-8603, Japan

    Wataru Shimizu MD, PhD

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  1. Wataru Shimizu MD, PhD
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Correspondence to Wataru Shimizu MD, PhD .

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  1. , Department of Internal Medicine, UMDNJ-RWJ Medical School, Robert Wood Johnson Pl. 1, New Brunswick, 08903-0019, New Jersey, USA

    Ihor Gussak

  2. , Gordon K. Moe Scholar, Masonic Medical Research Laboratory, Bleecker Street, Utica, 13501-1787, New York, USA

    Charles Antzelevitch

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Shimizu, W. (2013). Acquired Form of Brugada Syndrome. In: Gussak, I., Antzelevitch, C. (eds) Electrical Diseases of the Heart. Springer, London. https://doi.org/10.1007/978-1-4471-4978-1_7

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