Leviev Heart Institute, The Chaim Sheba Medical Center, Tel Hashomer, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
L'institut du Thorax, Service de Cardiologie, CHU de Nantes, Nantes, France; European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN GUARDHEART).
Heart Rhythm. 2019 Oct;16(10):1468-1474. doi: 10.1016/j.hrthm.2019.07.003. Epub 2019 Jul 5.
There is limited information on ethnic differences between patients with Brugada syndrome (BrS) and arrhythmic events (AEs).
The purpose of this study was to compare clinical, electrocardiographic (ECG), electrophysiological, and genetic characteristics between white and Asian patients with BrS and AEs.
The Survey on Arrhythmic Events in Brugada Syndrome is a multicenter survey from Western and Asian countries, gathering 678 patients with BrS and 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.
There was no difference in AE age onset (41.3 ± 16.1 years in whites vs 43.3 ± 12.3 years in Asians; P = .285). Higher proportions of whites were observed in pediatric and elderly populations. Asians were predominantly men (98.1% vs 85.7% in whites; P < .001) and frequently presented with aborted cardiac arrest (71.1% vs 56%; P < .001). Asians tended to display more spontaneous type 1 BrS-ECG pattern (71.5% vs 64.3%; P = .068). A family history of sudden cardiac death was noted more in whites (29.1% vs 11.5%; P < .001), with a higher rate of SCN5A mutation carriers (40.1% vs 13.2% in Asians; P < .001), as well as more fever-related AEs (8.5% vs 2.9%; P = .011). No difference was observed between the 2 groups regarding history of syncope and ventricular arrhythmia inducibility.
There are important differences between Asian and white patients with BrS. Asian patients present almost exclusively as male adults, more often with aborted cardiac arrest and spontaneous type 1 BrS-ECG. However, they have less family history of sudden cardiac death and markedly lower SCN5A mutation rates. The striking difference in SCN5A mutation rates should be tested in future studies.
Brugada 综合征(BrS)患者的种族差异相关信息有限,心律失常事件(AEs)也存在类似情况。
本研究旨在比较白种人和亚洲人种 BrS 伴 AEs 患者的临床、心电图(ECG)、电生理和遗传特征。
Brugada 综合征心律失常事件调查(Survey on Arrhythmic Events in Brugada Syndrome)是一项来自西方国家和亚洲国家的多中心调查,共纳入 678 例 BrS 伴首次记录的 AE 患者。排除其他(n = 14[2.1%])或未知(n = 30[4.4%])种族的患者后,纳入 364 例白种人和 270 例亚洲人种患者。
AE 发病年龄无差异(白人组为 41.3 ± 16.1 岁,亚洲人种组为 43.3 ± 12.3 岁;P =.285)。儿科和老年人群中白人占比更高。亚洲人种患者均为男性(98.1% vs 85.7%;P <.001),更常发生心搏骤停(71.1% vs 56%;P <.001)。亚洲人种更常出现自发 1 型 Brugada 心电图(ECG)模式(71.5% vs 64.3%;P =.068)。白人有家族性心源性猝死史(29.1% vs 11.5%;P <.001),SCN5A 基因突变携带者更多(40.1% vs 13.2%;P <.001),发热相关 AE 也更多(8.5% vs 2.9%;P =.011)。两组晕厥和心室心律失常诱发性史无差异。
BrS 患者中,白种人和亚洲人种存在重要差异。亚洲人种患者几乎均为成年男性,更常发生心搏骤停和自发 1 型 Brugada 心电图。然而,他们的家族性心源性猝死史较少,SCN5A 基因突变率显著更低。未来研究中应验证 SCN5A 基因突变率的明显差异。