Abstract
Brugada Syndrome is an inherited disease affecting ion channels of the heart leading to arrhythmia and sudden cardiac death. Brugada Syndrome typically manifests in middle age men after or during periods of rest, with the vast majority of sudden death occurring in individuals without structural heart defects. Cardiac ion channel dysfunction in Brugada Syndrome is well recognized; however, how voltage-gated calcium channel genes, specifically CACNA1C, CACNB2b, and CACNA2D1 are implicated, is a recent development. This chapter addresses why VGCCs are important for the ventricular action potential and how Brugada Syndrome is visualized on ECG, in particular the two types of elevated ST segments commonly used for clinical diagnosis. We also examine voltage-gated calcium channel mutations linked to Brugada Syndrome and how specific mutations cause a loss-of-function of the channel complex.
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Simms, B. (2014). Brugada Syndrome and Voltage-Gated Calcium Channels. In: Weiss, N., Koschak, A. (eds) Pathologies of Calcium Channels. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-40282-1_12
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