Brugada Syndrome: Clinical Care Amidst Pathophysiological Uncertainty

Julia C Isbister; Andrew D Krahn; Christopher Semsarian; Raymond W Sy

doi:10.1016/j.hlc.2019.11.016

Brugada Syndrome: Clinical Care Amidst Pathophysiological Uncertainty

Heart Lung Circ. 2020 Apr;29(4):538-546. doi: 10.1016/j.hlc.2019.11.016. Epub 2019 Dec 17.

Authors

Julia C Isbister¹, Andrew D Krahn², Christopher Semsarian¹, Raymond W Sy³

Affiliations

¹ Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, University of Sydney, Sydney, NSW, Australia; Faculty of Medicine and Health, University of Sydney, Sydney, NSW, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
² Division of Cardiology, University of British Columbia, Vancouver, Canada.
³ Faculty of Medicine and Health, University of Sydney, Sydney, NSW, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia. Electronic address: raymond.sy01@gmail.com.

PMID: 31959551
DOI: 10.1016/j.hlc.2019.11.016

Abstract

Brugada syndrome (BrS) is a complex clinical entity with ongoing conjecture regarding its genetic basis, underlying pathophysiology, and clinical management. Within this paradigm of uncertainty, clinicians are faced with the challenge of caring for patients with this uncommon but potentially fatal condition. This article reviews the current understanding of BrS and highlights the "known unknowns" to reinforce the need for flexible clinical practice in parallel with ongoing scientific discovery.

Keywords: Arrhythmogenesis; Brugada syndrome; Implantable cardioverter-defibrillator; Non-device management; Risk stratification; Sudden death.

Publication types

Review

MeSH terms

Brugada Syndrome* / diagnosis
Brugada Syndrome* / genetics
Brugada Syndrome* / physiopathology
Brugada Syndrome* / therapy
Humans
Risk Assessment
Risk Factors