Clinical and electrocardiographic characteristics of carriers with SCN5A mutations and non-SCN5A mutations in fever-induced Brugada syndrome
10.3760/cma.j.cn112148-20240927-00423
- VernacularTitle:发热诱发Brugada综合征中SCN5A变异及非SCN5A变异携带者的临床和心电图特征
- Author:
Keqin SHEN
1
;
Zhonghe ZHANG
;
Ganxiao CHEN
;
Xiaoxiong LIU
;
Guohua FAN
;
Jinqiu LIU
;
Hao XIA
;
Hong JIANG
;
Dan HU
Author Information
1. 武汉大学人民医院心血管内科及心血管病研究所 湖北省心脏重点实验室，武汉 430060
- Keywords:
Brugada syndrome;
Febrile;
Sudden cardiac death;
Genetics;
Electrophysiology
- From:
Chinese Journal of Cardiology
2024;52(12):1377-1382
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the differences in clinical and electrocardiographic characteristics between carriers of SCN5A mutations and non-SCN5A mutations in fever-induced Brugada syndrome.Methods:This study is a retrospective cohort study. A total of 263 patients with fever-induced Brugada syndrome who were admitted to Renmin Hospital of Wuhan University from January 2000 to December 2023 were selected. Their clinical manifestations, electrocardiographic characteristics, and major adverse cardiovascular events (MACE) at the time of diagnosis and during the follow-up period were collected. Among them, 200 patients underwent next-generation sequencing. Based on the genetic variation results, after excluding other mutations, they were divided into SCN5A mutation group, non-SCN5A sodium-related mutation group, potassium/calcium mutation group, and no mutation group. Comparisons were made among these groups in terms of their clinical and electrocardiographic characteristics.Results:Among the 263 patients with fever-induced Brugada syndrome, the mean age was (41.9±17.6) years, with 80.6% (212/263) being male. The median follow-up duration was 53.0 months, and 13.7% (36/263) of the patients experienced MACE. The rate of SCN5A mutation was 34.5% (69/200), while the rates of non-SCN5A sodium-related mutations and potassium/calcium-related mutations were 4.5% (9/200) and 3.5% (7/200), respectively. The SCN5A mutation group was younger than the non-SCN5A sodium-related mutation group and the no mutation group (ages were (33.8±14.7), (49.8±11.6), (44.6±15.7) years, respectively, P<0.001). The SCN5A mutation group also had a longer PR interval than the no mutation group ((176.8±32.3) ms vs. (163.9±28.6) ms, P=0.034). The incidence of MACE was higher in the non-SCN5A sodium-related mutation group than that in the no mutation group (55.6% (5/9) vs. 9.1% (9/99), P=0.002). Conclusions:Fever-induced Brugada syndrome patients carrying non-SCN5A mutations exhibit distinct clinical and electrocardiographic characteristics compared to those with SCN5A mutations. These differences warrant attention in clinical practice.
