Relations between right ventricular morphology and clinical, electrical and genetic parameters in Brugada Syndrome

Relations between right ventricular morphology and clinical, electrical and genetic parameters in Brugada Syndrome.

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Item Type: Article
Status: Published
Official URL:
Journal or Publication Title: PLOS ONE
Volume: 13
Number: 4
Page Range: e0195594
Date: 2018
Divisions: Bioinformatics and Molecular Genetics
Cardio Genomics
Molecular Cardiology
Depositing User: General Admin
Identification Number: 10.1371/journal.pone.0195594
ISSN: 1932-6203
Date Deposited: 03 Jan 2021 22:20

Background: Increasing evidence suggests the presence of structural changes affecting the right ventricular outflow tract (RVOT) in patients with Brugada Syndrome (BrS). The aim of this study was to characterise the RV morphology in BrS and explore associations between morphologic, clinical, electrical, and genetic parameters using non-invasive multimodality testing.

Methods: Consecutive BrS patients (recruited 2013-2015) underwent clinical assessment, dedicated RV imaging using cardiac magnetic resonance (CMR) imaging (unless contra-indicated), electrical assessment (electrocardiogram, Holter monitoring, signal-averaged ECG[SAECG]) and genotyping. Morphologic data were compared to matched control and unmatched ARVC (arrhythmogenic right ventricular cardiomyopathy) cohorts, and potential associations between morphologic parameters and other variables were explored.

Results: BrS patients (n = 42, male 86%, age 46±12 years) exhibited normal global RV volume and function, comparable to control, in contrast to significantly larger, impaired RVs in ARVC cohort (RVESV p = 0.0001; RVEDV p<0.0001, RVEF p = 0.002). Compared with control, BrS patients exhibited larger RVOT volumes (7.4 ± 0.7 vs 5.8 ± 0.7 mL/m2, p<0.0001) and wall motion abnormalities (RWMA) (31% vs 0%, p = 0.005); compared with ARVC cohort, the RVOT volumes were similar (7.4 ± 0.7 vs, 8.1 ± 1.7, p = 0.52) and there were less RWMA (31% vs 76%, p = 0.01). Overall 67% BrS patients had abnormal RVOT morphology. Patients with abnormal RVOT tended to be older (48 ± 12 y vs 41 ± 12y, p = 0.06). Rare genetic variants were only observed in patients with abnormal RVOT morphology (36% vs 0%, p = 0.02).

Conclusions: Patients with BrS frequently exhibit structural abnormalities localised to the RVOT and these changes may be age- and gene-dependent.

Ai, Tomohiko
Gray, Belinda
Gnanappa, Ganesh Kumar
Bagnall, Richard D.
Femia, Giuseppe
Yeates, Laura
Ingles, Jodie
Burns, Charlotte
Puranik, Rajesh
Grieve, Stuart M.
Semsarian, Christopher
Sy, Raymond W.
Last Modified: 03 Jan 2021 22:20

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