背景:患有Brugada综合征(BrS)的年轻(<18岁)患者在BrS研究及其管理中的代表性往往不足,尤其是与晕厥发作有关,尚不清楚。
目的:本研究旨在描述通过植入式环形记录仪(ILR)进行连续节律监测的年轻BrS患者的心律失常患病率,并评估原因不明的晕厥的病因。
方法:在12个国际中心共纳入147例患有ILR的BrS患者,分为儿科(<12岁;n=77,52%)和青少年(13-18岁;n=70,48%)。
结果:平均年龄为11.3岁,53例(36.1%)为女性,31例(21.1%)有自发性1型心电图。中位随访时间为3.6年(Q1-Q3:1.6-4.8年),33例患者(22.4%)发生心律失常事件,主要为非心室起源:15例心房事件(10.2%)和16例缓慢性心律失常事件(10.9%)。4例患者发生室性心律失常,都是自发的BrS,一半与发烧有关。在随访期间所有晕厥复发的患者中,真正的心律失常性晕厥被记录在5(17.8%),3例(60%)是由于心律失常缓慢或房性心律失常。
结论:在年轻的BrS患者中使用ILRs进行连续心律监测可发现广泛的心律失常。室性心律失常主要发生在自发性1型心电图患者和发热期间。尽管年纪小,在60%的患者中,缓慢心律失常和房性心律失常是常见的,是心律失常性晕厥的原因。患有不明原因晕厥的年轻BrS患者可能会从ILR植入物中受益。
BACKGROUND: Young (<18 years of age) patients with Brugada syndrome (BrS) are often under-represented in BrS studies and their management, especially related to syncopal episodes, remains unclear.
OBJECTIVE: This study sought to describe the arrhythmia prevalence among young patients with BrS undergoing continuous rhythm monitoring by implantable loop recorder (ILR) and to assess the etiology behind syncope of undetermined origin.
METHODS: A total of 147 patients with BrS with ILR were enrolled in 12 international centers and divided into pediatric (age <12 years; n = 77, 52%) and adolescents (age 13-18 years; n = 70, 48%).
RESULTS: Mean age was 11.3 years, 53 patients (36.1%) were female, and 31 (21.1%) had spontaneous type 1 electrocardiograms. Over a median follow-up of 3.6 years (Q1-Q3: 1.6-4.8 years), an arrhythmic event was recorded in 33 patients (22.4%), mainly of nonventricular origin: 15 atrial (10.2%) and 16 bradyarrhythmic events (10.9%). Ventricular arrhythmias occurred in 4 patients, all with spontaneous BrS, and were fever-related in one-half. Among all patients with recurrence of syncope during follow-up, true arrhythmic syncope was documented in 5 (17.8%), and it was due to bradyarrhythmias or atrial arrhythmias in 3 cases (60%).
CONCLUSIONS: Continuous rhythm monitoring with ILRs in young patients with BrS detects a broad range of arrhythmias. Ventricular arrhythmias occur predominantly in patients with spontaneous type 1 electrocardiograms and during fever. Despite the young age, bradyarrhythmias and atrial arrhythmias are frequent and represent the cause of arrhythmic syncope in 60% of patients. Young patients with BrS with syncope of undetermined origin may benefit from ILR implant.