Ethnic Differences in Patients with Brugada Syndrome and Arrhythmic Events: New Insights from SABRUS

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Anat Milman, Antoine Andorin, Pieter G Postema, Jean-Baptiste Gourraud, Frederic Sacher, Philippe Mabo, Sung-Hwan Kim, Shingo Maeda, Yoshihide Takahashi, Tsukasa Kamakura, Takeshi Aiba, Giulio Conte, Jimmy Jm Juang, Eran Leshem, Yoav Michowitz, Rami Fogelman, Aviram Hochstadt, Yuka Mizusawa, Carla Giustetto, Elena Arbelo & 27 others Zhengrong Huang, Domenico Corrado, Pietro Delise, Giuseppe Allocca, Masahiko Takagi, Yanushi D Wijeyeratne, Andrea Mazzanti, Ramon Brugada, Ruben Casado-Arroyo, Jean Champagne, Leonardo Calo, Georgia Sarquella-Brugada, Camilla H Jespersen, Jacob Tfelt-Hansen, Christian Veltmann, Silvia G Priori, Elijah R Behr, Gan-Xin Yan, Josep Brugada, Fiorenzo Gaita, Arthur A M Wilde, Pedro Brugada, Kengo F Kusano, Kenzo Hirao, Gi-Byoung Nam, Vincent Probst, Bernard Belhassen

BACKGROUND: There is limited information on ethnic differences between patients with Brugada syndrome (BrS) with arrhythmic events (AEs).

OBJECTIVES: To compare clinical, electrocardiographic (ECG), electrophysiologic (EP) and genetic characteristics between White and Asian BrS-patients with AE.

METHODS: SABRUS is a multicenter survey from Western and Asian countries, gathering 678 BrS-patients with first documented AE. After excluding patients with other (n=14; 2.1%) or unknown (n=30; 4.4%) ethnicity, 364 (53.7%) Whites and 270 (39.8%) Asians comprised the study group.

RESULTS: There was no difference in AE age onset (41.3±16.1 years in Whites vs. 43.3±12.3 years in Asians, P=0.285). Higher proportions of Whites were observed in pediatric and elderly populations. Asians were predominantly males (98.1% vs. 85.7% in Whites, P<0.001) and frequently presented with aborted cardiac arrest (ACA) (71.1% vs. 56%, P<0.001). Asians tended to display more spontaneous type 1 BrS-ECG (71.5% vs. 64.3%, P=0.068). Family history of sudden cardiac death (FHSCD) was noted more in Whites (29.1% vs. 11.5%, P<0.001), with higher rate of SCN5A mutation carriers (40.1% vs. 13.2% in Asians, P<0.001), as well as more fever-related AEs (8.5% vs. 2.9%, 0.011). No difference was observed between the two groups regarding prior history of syncope and ventricular arrhythmia inducibility.

CONCLUSIONS: There are important differences between Asian and White BrS-patients. Asian patients present almost exclusively as male adults, more often with ACA and spontaneous type 1 BrS-ECG. However, they have less FHSCD and markedly lower SCN5A mutation rates. The striking difference in SCN5A mutation rates should be tested in future studies.

Original languageEnglish
JournalHeart Rhythm
Publication statusE-pub ahead of print - 5 Jul 2019

ID: 225424223