Longitudinal assessment of structural phenotype in Brugada syndrome using cardiac magnetic resonance imaging

Julia C. Isbister, MBBS; Belinda Gray, BSc (Med), MBBS; Sophie Offen, BSc, MBBS; Laura Yeates, BSc (hons), GradDipGenCouns; Chris Naoum, MBBS, PhD; Caroline Medi, BMed, PhD; Hariharan Raju, MBChB, PhD; Christopher Semsarian, MBBS, PhD, MPH, FHRS; Rajesh Puranik, MBBS, PhD; Raymond W. Sy, MBBS, PhD, FHRS

Heart Rhythm O2 (Jan 2023)

Longitudinal assessment of structural phenotype in Brugada syndrome using cardiac magnetic resonance imaging

Julia C. Isbister, MBBS,
Belinda Gray, BSc (Med), MBBS,
Sophie Offen, BSc, MBBS,
Laura Yeates, BSc (hons), GradDipGenCouns,
Chris Naoum, MBBS, PhD,
Caroline Medi, BMed, PhD,
Hariharan Raju, MBChB, PhD,
Christopher Semsarian, MBBS, PhD, MPH, FHRS,
Rajesh Puranik, MBBS, PhD,
Raymond W. Sy, MBBS, PhD, FHRS

Affiliations

Julia C. Isbister, MBBS: Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, University of Sydney, Sydney, New South Wales, Australia; Faculty of Medicine and Heath, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
Belinda Gray, BSc (Med), MBBS: Faculty of Medicine and Heath, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
Sophie Offen, BSc, MBBS: Faculty of Medicine and Heath, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
Laura Yeates, BSc (hons), GradDipGenCouns: Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, University of Sydney, Sydney, New South Wales, Australia; Faculty of Medicine and Heath, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Centre for Population Genomics, Garvan Institute of Medical Research, University of New South Wales, Sydney, New South Wales, Australia; Centre for Population Genomics, Murdoch Children’s Research Institute, Melbourne, Victoria, Australia
Chris Naoum, MBBS, PhD: Department of Cardiology, Concord Repatriation General Hospital, Sydney, New South Wales, Australia
Caroline Medi, BMed, PhD: Faculty of Medicine and Heath, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
Hariharan Raju, MBChB, PhD: Faculty of Medicine and Heath, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Concord Repatriation General Hospital, Sydney, New South Wales, Australia; Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, New South Wales, Australia
Christopher Semsarian, MBBS, PhD, MPH, FHRS: Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, University of Sydney, Sydney, New South Wales, Australia; Faculty of Medicine and Heath, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
Rajesh Puranik, MBBS, PhD: Faculty of Medicine and Heath, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
Raymond W. Sy, MBBS, PhD, FHRS: Faculty of Medicine and Heath, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Department of Cardiology, Concord Repatriation General Hospital, Sydney, New South Wales, Australia; Address reprint requests and correspondence: Dr Raymond W. Sy, University of Sydney, Camperdown, NSW, 2006, Australia.

Journal volume & issue: Vol. 4, no. 1
pp. 34 – 41

Abstract

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Background: Despite historically being considered a channelopathy, subtle structural changes have been reported in Brugada syndrome (BrS) on histopathology and cardiac magnetic resonance (CMR) imaging. It is not known if these structural changes progress over time. Objective: The study sought to assess if structural changes in BrS evolve over time with serial CMR assessment and to investigate the utility of parametric mapping techniques to identify diffuse fibrosis in BrS. Methods: Patients with a diagnosis of BrS based on international guidelines and normal CMR at least 3 years prior to the study period were invited to undergo repeat CMR. CMR images were analyzed de novo and compared at baseline and follow-up. Results: Eighteen patients with BrS (72% men; mean age at follow-up 47.4 ± 8.9 years) underwent serial CMR with an average of 5.0 ± 1.7 years between scans. No patients had late gadolinium enhancement (LGE) on baseline CMR, but 4 (22%) developed LGE on follow-up, typically localized to the right ventricular (RV) side of the basal septum. RV end-systolic volume increased over time (P = .04) and was associated with a trend toward reduction in RV ejection fraction (P = .07). Four patients showed a reduction in RV ejection fraction >10%. There was no evidence of diffuse myocardial fibrosis observed on parametric mapping. Conclusions: Structural changes may evolve over time with development of focal fibrosis, evidenced by LGE on CMR in a significant proportion of patients with BrS. These findings have implications for our understanding of the pathological substrate in BrS and the longitudinal evaluation of patients with BrS.

Published in Heart Rhythm O2

ISSN: 2666-5018 (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the circulatory (Cardiovascular) system
Website: https://www.journals.elsevier.com/heart-rhythm-o2

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