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

Rachel Bastiaenen, Andrew T. Cox, Silvia Castelletti, Yanushi D. Wijeyeratne, Nicholas Colbeck, Nadia Pakroo, Hammad Ahmed, Nick Bunce, Lisa Anderson, James C. Moon, Sanjay Prasad, Sanjay Sharma, Elijah R. Behr

Research output: Contribution to journalArticle

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Abstract

Background There is increasing evidence that the Brugada ECG pattern is a marker of subtle structural heart disease. Objective The purpose of this study was to characterize patients with Brugada syndrome (BrS) using cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). Methods BrS was diagnosed according to international guidelines. Twenty-six percent of patients with BrS carried SCN5A mutations. CMR data from 78 patients with BrS were compared with 78 healthy controls (44 ± 15 vs 42 ± 14 years; P =.434; and 64% vs 64% male; P = 1). Results Right ventricular (RV) ejection fraction was slightly lower (61 ± 8% vs 64 ± 5%; P =.004) and RV end-systolic volume slightly greater (31 ± 10 mL/m2 vs 28 ± 6 mL/m2; P =.038) in BrS compared with controls. These values remained within the normal range. LGE was demonstrated in 8% of patients with BrS (left ventricular midwall LGE in 5%) but not in controls (P =.028). In patients with BrS with midwall LGE there were no other features of cardiomyopathy at the time of CMR, but genetic testing and follow-up revealed a desmoplakin mutation in 1 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 patients with BrS have left ventricular 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.

Original languageEnglish
Pages (from-to)583-589
Number of pages7
JournalHeart Rhythm
Volume14
Issue number4
DOIs
Publication statusPublished - Apr 1 2017

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Brugada Syndrome
Gadolinium
Cardiomyopathies
Magnetic Resonance Spectroscopy
Stroke Volume
Desmoplakins
Arrhythmogenic Right Ventricular Dysplasia
Right Ventricular Function
Mutation
Genetic Testing
Heart Diseases
Electrocardiography

Keywords

  • Arrhythmogenic right ventricular cardiomyopathy
  • Brugada syndrome
  • Cardiac magnetic resonance
  • Cardiomyopathy
  • Late gadolinium enhancement

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Bastiaenen, R., Cox, A. T., Castelletti, S., Wijeyeratne, Y. D., Colbeck, N., Pakroo, N., ... Behr, E. R. (2017). Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying cardiomyopathy? Heart Rhythm, 14(4), 583-589. https://doi.org/10.1016/j.hrthm.2016.12.004

Late gadolinium enhancement in Brugada syndrome : A marker for subtle underlying cardiomyopathy? / Bastiaenen, Rachel; Cox, Andrew T.; Castelletti, Silvia; Wijeyeratne, Yanushi D.; Colbeck, Nicholas; Pakroo, Nadia; Ahmed, Hammad; Bunce, Nick; Anderson, Lisa; Moon, James C.; Prasad, Sanjay; Sharma, Sanjay; Behr, Elijah R.

In: Heart Rhythm, Vol. 14, No. 4, 01.04.2017, p. 583-589.

Research output: Contribution to journalArticle

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, vol. 14, no. 4, pp. 583-589. https://doi.org/10.1016/j.hrthm.2016.12.004
Bastiaenen, Rachel ; Cox, Andrew T. ; Castelletti, Silvia ; Wijeyeratne, Yanushi D. ; Colbeck, Nicholas ; Pakroo, Nadia ; Ahmed, Hammad ; Bunce, Nick ; Anderson, Lisa ; Moon, James C. ; Prasad, Sanjay ; Sharma, Sanjay ; Behr, Elijah R. / Late gadolinium enhancement in Brugada syndrome : A marker for subtle underlying cardiomyopathy?. In: Heart Rhythm. 2017 ; Vol. 14, No. 4. pp. 583-589.
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abstract = "Background There is increasing evidence that the Brugada ECG pattern is a marker of subtle structural heart disease. Objective The purpose of this study was to characterize patients with Brugada syndrome (BrS) using cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). Methods BrS was diagnosed according to international guidelines. Twenty-six percent of patients with BrS carried SCN5A mutations. CMR data from 78 patients with BrS were compared with 78 healthy controls (44 ± 15 vs 42 ± 14 years; P =.434; and 64{\%} vs 64{\%} male; P = 1). Results Right ventricular (RV) ejection fraction was slightly lower (61 ± 8{\%} vs 64 ± 5{\%}; P =.004) and RV end-systolic volume slightly greater (31 ± 10 mL/m2 vs 28 ± 6 mL/m2; P =.038) in BrS compared with controls. These values remained within the normal range. LGE was demonstrated in 8{\%} of patients with BrS (left ventricular midwall LGE in 5{\%}) but not in controls (P =.028). In patients with BrS with midwall LGE there were no other features of cardiomyopathy at the time of CMR, but genetic testing and follow-up revealed a desmoplakin mutation in 1 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 patients with BrS have left ventricular 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.",
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AU - Colbeck, Nicholas

AU - Pakroo, Nadia

AU - Ahmed, Hammad

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N2 - Background There is increasing evidence that the Brugada ECG pattern is a marker of subtle structural heart disease. Objective The purpose of this study was to characterize patients with Brugada syndrome (BrS) using cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). Methods BrS was diagnosed according to international guidelines. Twenty-six percent of patients with BrS carried SCN5A mutations. CMR data from 78 patients with BrS were compared with 78 healthy controls (44 ± 15 vs 42 ± 14 years; P =.434; and 64% vs 64% male; P = 1). Results Right ventricular (RV) ejection fraction was slightly lower (61 ± 8% vs 64 ± 5%; P =.004) and RV end-systolic volume slightly greater (31 ± 10 mL/m2 vs 28 ± 6 mL/m2; P =.038) in BrS compared with controls. These values remained within the normal range. LGE was demonstrated in 8% of patients with BrS (left ventricular midwall LGE in 5%) but not in controls (P =.028). In patients with BrS with midwall LGE there were no other features of cardiomyopathy at the time of CMR, but genetic testing and follow-up revealed a desmoplakin mutation in 1 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 patients with BrS have left ventricular 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.

AB - Background There is increasing evidence that the Brugada ECG pattern is a marker of subtle structural heart disease. Objective The purpose of this study was to characterize patients with Brugada syndrome (BrS) using cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). Methods BrS was diagnosed according to international guidelines. Twenty-six percent of patients with BrS carried SCN5A mutations. CMR data from 78 patients with BrS were compared with 78 healthy controls (44 ± 15 vs 42 ± 14 years; P =.434; and 64% vs 64% male; P = 1). Results Right ventricular (RV) ejection fraction was slightly lower (61 ± 8% vs 64 ± 5%; P =.004) and RV end-systolic volume slightly greater (31 ± 10 mL/m2 vs 28 ± 6 mL/m2; P =.038) in BrS compared with controls. These values remained within the normal range. LGE was demonstrated in 8% of patients with BrS (left ventricular midwall LGE in 5%) but not in controls (P =.028). In patients with BrS with midwall LGE there were no other features of cardiomyopathy at the time of CMR, but genetic testing and follow-up revealed a desmoplakin mutation in 1 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 patients with BrS have left ventricular 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.

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KW - Cardiomyopathy

KW - Late gadolinium enhancement

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