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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; Bunce, N; Anderson, L; Moon, JC; et al. Bastiaenen, R; Cox, AT; Castelletti, S; Wijeyeratne, YD; Colbeck, N; Pakroo, N; Ahmed, H; Bunce, N; Anderson, L; Moon, JC; Prasad, S; Sharma, S; Behr, ER (2017) Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying cardiomyopathy? Heart Rhythm, 14 (4). pp. 583-589. ISSN 1556-3871 https://doi.org/10.1016/j.hrthm.2016.12.004
SGUL Authors: Bastiaenen, Rachel Marie Behr, Elijah Raphael Anderson, Lisa Sharma, Sanjay Wijeyeratne, Yanushi Dullewe

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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.

Item Type: Article
Additional Information: © 2016. 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, Cardiovascular System & Hematology, 1102 Cardiovascular Medicine And Haematology, 0903 Biomedical Engineering
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cardiac (INCCCA)
Journal or Publication Title: Heart Rhythm
ISSN: 1556-3871
Language: eng
Dates:
DateEvent
April 2017Published
3 December 2016Published Online
28 September 2016Accepted
Publisher License: Creative Commons: Attribution-Noncommercial-No Derivative Works 4.0
Projects:
Project IDFunderFunder ID
UNSPECIFIEDNational Institute for Health Researchhttp://dx.doi.org/10.13039/501100000272
PubMed ID: 27919765
Go to PubMed abstract
URI: http://openaccess.sgul.ac.uk/id/eprint/108387
Publisher's version: https://doi.org/10.1016/j.hrthm.2016.12.004

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