Brugada syndrome (BrS) has been traditionally considered a pure electrical disorder without an underlying structural substrate. However, early ECG studies showed the presence of depolarization abnormalities in this condition, while many studies based on advanced imaging have suggested the presence of subtle structural alterations. On the other hand, electrophysiological study (EPS) and electroanatomic mapping (EAM) techniques have provided important data regarding right ventricular functional and structural arrhythmic substrate. More recently, histology and immunology shed light on the possible role of fibrotic and inflammatory substrates in BrS. Notably, a significant overlap between electro anatomical and structural features in BrS and arrhythmogenic cardiomyopathy has been proposed. In this review, we summarized the physio pathological pathways and substrate underlying BrS.
Brugada Syndrome: Channelopathy and/or Cardiomyopathy / Ciabatti M., Notarstefano P., Zocchi C., Virgili G., Bellocci F., Olivotto I., Pieroni M.. - In: CARDIOGENETICS. - ISSN 2035-8253. - ELETTRONICO. - 15:(2025), pp. 17.0-17.0. [10.3390/cardiogenetics15020017]
Brugada Syndrome: Channelopathy and/or Cardiomyopathy
Zocchi C.;Olivotto I.;Pieroni M.
2025
Abstract
Brugada syndrome (BrS) has been traditionally considered a pure electrical disorder without an underlying structural substrate. However, early ECG studies showed the presence of depolarization abnormalities in this condition, while many studies based on advanced imaging have suggested the presence of subtle structural alterations. On the other hand, electrophysiological study (EPS) and electroanatomic mapping (EAM) techniques have provided important data regarding right ventricular functional and structural arrhythmic substrate. More recently, histology and immunology shed light on the possible role of fibrotic and inflammatory substrates in BrS. Notably, a significant overlap between electro anatomical and structural features in BrS and arrhythmogenic cardiomyopathy has been proposed. In this review, we summarized the physio pathological pathways and substrate underlying BrS.I documenti in FLORE sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.



