Cardiocentro Ticino Institute, Ente Ospedaliero Cantonale, Lugano, Switzerland.
Arrhythmology Department, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Donato, Milan, Italy; School of Medicine, University Vita-Salute San Raffaele, Milan, Italy.
J Am Coll Cardiol. 2024 Sep 3;84(10):921-933. doi: 10.1016/j.jacc.2024.04.070.
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.
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.
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%).
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%).
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.
患有布鲁加达综合征（BrS）的年轻（<18 岁）患者在 BrS 研究和管理中往往代表性不足，特别是与晕厥发作相关的管理仍不清楚。
本研究旨在描述通过植入式环路记录器（ILR）对年轻 BrS 患者进行连续节律监测的心律失常发生率，并评估不明原因晕厥的病因。
总共纳入 12 个国际中心的 147 名 BrS 患者并植入 ILR，分为儿科（年龄<12 岁；n=77，52%）和青少年（年龄 13-18 岁；n=70，48%）。
平均年龄为 11.3 岁，53 名患者（36.1%）为女性，31 名（21.1%）有自发 I 型心电图。在中位随访 3.6 年（Q1-Q3：1.6-4.8 年）期间，33 名患者（22.4%）记录到心律失常事件，主要是非室性起源：15 次房性（10.2%）和 16 次缓发性心律失常事件（10.9%）。4 名患者发生室性心律失常，均为自发性 BrS，半数与发热相关。在所有随访期间复发晕厥的患者中，有 5 例（17.8%）记录到真正的心律失常性晕厥，其中 3 例（60%）是由于缓发性心律失常或房性心律失常引起的。
在年轻的 BrS 患者中使用 ILR 进行连续节律监测可检测到广泛的心律失常。室性心律失常主要发生在自发性 I 型心电图和发热的患者中。尽管年龄较小，但缓发性心律失常和房性心律失常很常见，占心律失常性晕厥患者的 60%。不明原因晕厥的年轻 BrS 患者可能受益于 ILR 植入。
