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Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying cardiomyopathy?

Bastiaenen, R; Cox, AT; Castelletti, S; Wijeyeratne, YD; Colbeck, N; Pakroo, N; Ahmed, H; ... Behr, ER; + view all (2017) Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying cardiomyopathy? Heart Rhythm , 14 (4) pp. 583-589. 10.1016/j.hrthm.2016.12.004. Green open access

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Abstract

BACKGROUND: There is increasing evidence that the Brugada ECG pattern is a marker of subtle structural heart disease. OBJECTIVE: We characterised Brugada syndrome (BrS) patients using cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). METHODS: BrS was diagnosed according to international guidelines. 26% BrS patients carried SCN5A mutations. CMR data from 78 BrS patients were compared with 78 healthy controls (44±15 vs 42±14 years; p=0.434 and 64% vs 64% male; p=1.000). RESULTS: Right ventricular (RV) ejection fraction was slightly lower (61±8% vs 64±5%; p=0.004) and RV end-systolic volume slightly greater (31±10mL/m(2) vs 28±6mL/m(2); p=0.038) in BrS compared with controls. These values remained within the normal range. LGE was demonstrated in 8% BrS patients (left ventricular (LV) midwall LGE in 5%) but not in controls (p=0.028). In BrS patients with midwall LGE there were no other features of cardiomyopathy at the time of CMR but genetic testing and follow-up has revealed a desmoplakin mutation in one patient and evolution of T-wave inversion throughout all precordial ECG leads in another. Neither patient fulfils diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy. CONCLUSION: Some BrS patients have LV midwall LGE consistent with an underlying cardiomyopathic process. Even cases without LGE show greater RV volumes and reduced RV function. These findings lend further support to the presence of subtle structural abnormalities in BrS. The BrS pattern with LGE may serve as early markers for evolution of a cardiomyopathic phenotype over time. CMR is a potentially useful adjunct investigation in the clinical evaluation of BrS.

Type: Article
Title: Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying cardiomyopathy?
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.hrthm.2016.12.004
Publisher version: http://dx.doi.org/10.1016/j.hrthm.2016.12.004
Language: English
Additional information: Copyright © 2017 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/
Keywords: Arrhythmogenic right ventricular cardiomyopathy, Brugada syndrome, Cardiac magnetic resonance, Cardiomyopathy, Late gadolinium enhancement
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Clinical Science
URI: https://discovery.ucl.ac.uk/id/eprint/1531887
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