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Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying cardiomyopathy?
- Source :
- Heart Rhythm. 14:583-589
- Publication Year :
- 2017
- Publisher :
- Elsevier BV, 2017.
-
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.
- Subjects :
- Adult
Male
medicine.medical_specialty
Heart disease
Heart Ventricles
Cardiomyopathy
Contrast Media
Magnetic Resonance Imaging, Cine
Gadolinium
030204 cardiovascular system & hematology
Right ventricular cardiomyopathy
NAV1.5 Voltage-Gated Sodium Channel
030218 nuclear medicine & medical imaging
03 medical and health sciences
0302 clinical medicine
Physiology (medical)
Internal medicine
medicine
Humans
Late gadolinium enhancement
cardiovascular diseases
Brugada Syndrome
Brugada syndrome
Ejection fraction
medicine.diagnostic_test
biology
business.industry
Desmoplakin
fungi
Reproducibility of Results
Stroke Volume
Magnetic resonance imaging
Organ Size
Middle Aged
Image Enhancement
medicine.disease
Mutation
Cardiology
biology.protein
Female
Cardiomyopathies
Cardiology and Cardiovascular Medicine
business
Subjects
Details
- ISSN :
- 15475271
- Volume :
- 14
- Database :
- OpenAIRE
- Journal :
- Heart Rhythm
- Accession number :
- edsair.doi.dedup.....585263b6cd3be3d417d45ad5f475f486