Abstract
Background: Slow-conductive structural abnormalities located in the epicardium of the right ventricle underlie Brugada syndrome. This investigation examined whether similar substrate extends to the left ventricle (LV).
Methods and Results: Study population consisted of 22 patients (mean age 46 ± 11 years, predominantly male) experiencing recurrent ventricular arrhythmias. High-density biventricular epicardial mapping identified abnormal substrate in all participants’ right ventricular epicardium (27 ± 11 cm²) and in 45% of cases on the LV epicardium.
Key findings revealed that LV substrate patients exhibited longer arrhythmia histories, prolonged cardiac intervals, and higher rates of SCN5A mutations (70% versus 25%). Those with SCN5A mutations demonstrated elevated polygenic risk scores compared to unselected populations.
Conclusion: A subset of patients with Brugada syndrome present an abnormal substrate extending onto the LV epicardium and inferior RV that is associated with SCN5A mutations and multigenic variants.
Publication Information
- Journal: JACC Clinical Electrophysiology
- Volume: 9, Issue 10
- Pages: 2041-2051
- Publication Date: October 2023
- DOI: 10.1016/j.jacep.2023.05.039
- PMID: 37480873
- PubMed Link: https://pubmed.ncbi.nlm.nih.gov/37480873/
- JACC Link: https://www.jacc.org/doi/10.1016/j.jacep.2023.05.039
Authors
Ghassen Cheniti, Michel Haissaguerre, Christian Dina, Tsukasa Kamakura, Josselin Duchateau, Frederic Sacher, Hugo-Pierre Racine, Elodie Surget, Floriane Simonet, Jean-Baptiste Gourraud, Soumaya Sridi, Hubert Cochet, Clementine Andre, Benjamin Bouyer, Remi Chauvel, Romain Tixier, Nicolas Derval, Thomas Pambrun, Remi Dubois, Pierre Jais, Koonlawee Nademanee, Richard Redon, Jean-Jacques Schott, Vincent Probst, Meleze Hocini, Julien Barc, Olivier Bernus