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性别差异在 Brugada 综合征的临床和遗传特征:SADS-TW BrS 注册研究。

Gender difference in clinical and genetic characteristics of Brugada syndrome: SADS-TW BrS registry.

机构信息

Cardiovascular Center and Division of Cardiology, Department of Internal Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.

Division of Cardiology, Department of Internal Medicine, National Taiwan University Hospital Hsinchu Branch, Hsinchu City, Taiwan.

出版信息

QJM. 2019 May 1;112(5):343-350. doi: 10.1093/qjmed/hcz028.

DOI:10.1093/qjmed/hcz028
PMID:30690642
Abstract

BACKGROUND

Brugada syndrome (BrS) is a heritable sudden cardiac death (SCD) disease with male predominance. Information on gender difference of BrS remains scarce.

AIM

To investigate the gender difference of BrS in Han Chinese.

DESIGN

We consecutively enrolled 169 BrS patients (153 males and 16 females) from Han Chinese in Taiwan from 1998 to 2017.

METHODS

Clinical characteristics, electrocardiographic parameters and SCN5A mutation status were compared between genders.

RESULTS

The percentage of family history of SCD in females was slightly higher (31.3% vs. 15%, P = 0.15). Females exhibited longer QTc (457.8 ± 33.0 vs. 429.5 ± 42.1 ms, P < 0.01). Regarding cumulative event occurrence by age, Mantel-Cox test showed females had earlier age of onset of first cardiac events (SCD or syncope) than males (P = 0.049), which was mainly attributed to syncope (P < 0.01). Males with SCD exhibited longer QRS duration (114.2 ± 26.8 vs. 104.8 ± 15.3 ms, P = 0.02) and QTc (442.5 ± 57.4 vs. 422.9 ± 28.8 ms, P = 0.02). Males with syncope exhibited longer PR interval (181.2 ± 33.7 vs. 165.7 ± 27.1 ms, P = 0.01), whereas females with SCD or syncope had a trend towards slower heart rates (69.1 ± 9.6 vs. 82.2 ± 16.3 bpm, P = 0.10) than female with no or mild symptoms. There was no difference in the percentage of SCN5A mutation between genders.

CONCLUSION

Gender difference is present in BrS. Females have longer QTc and suffer from syncope earlier than males. Risk of SCD in males is associated with boarder QRS complex and longer QTc, whereas risk of syncope is associated with longer PR interval in males and slower heart rate in females.

摘要

背景

Brugada 综合征(BrS)是一种遗传性心源性猝死(SCD)疾病,以男性为主。关于 BrS 的性别差异信息仍然很少。

目的

探讨汉族人群中 BrS 的性别差异。

设计

我们连续纳入了 1998 年至 2017 年来自中国台湾汉族的 169 名 BrS 患者(男性 153 例,女性 16 例)。

方法

比较了不同性别之间的临床特征、心电图参数和 SCN5A 突变状态。

结果

女性家族性 SCD 病史的比例略高(31.3% vs. 15%,P=0.15)。女性的 QTc 更长(457.8±33.0 vs. 429.5±42.1 ms,P<0.01)。通过 Mantel-Cox 检验,在按年龄累积事件发生方面,女性首发心脏事件(SCD 或晕厥)的年龄早于男性(P=0.049),主要是晕厥(P<0.01)。SCD 组男性 QRS 波时限(114.2±26.8 vs. 104.8±15.3 ms,P=0.02)和 QTc(442.5±57.4 vs. 422.9±28.8 ms,P=0.02)更长。晕厥组男性 PR 间期更长(181.2±33.7 vs. 165.7±27.1 ms,P=0.01),而 SCD 或晕厥组女性心率较慢(69.1±9.6 vs. 82.2±16.3 bpm,P=0.10)。男女之间 SCN5A 突变的百分比没有差异。

结论

BrS 存在性别差异。女性的 QTc 较长,且比男性更早发生晕厥。男性 SCD 风险与更宽的 QRS 波复合体和更长的 QTc 相关,而男性晕厥风险与更长的 PR 间期和女性更慢的心率相关。

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