Abstract：
Brugada syndrome (BrS) is a rare inherited arrhythmia syndrome. Affected children may experience life-threatening symptoms, mainly during fever. The percentage of SCN5A variant carriers in children is higher than in adults. Current diagnostic and follow-up policies for children with (a family history of) BrS vary between centres. Here, we present a consensus statement based on the current literature and expert opinions to standardise the approach for all children with BrS and those from BrS families in the Netherlands. In summary, BrS is diagnosed in patients with a spontaneous type 1 electrocardiogram (ECG) pattern or with a Shanghai score ≥ 3.5 including ≥ 1 ECG finding. A sodium channel-blocking drug challenge test should only be performed after puberty with a few exceptions. A fever ECG is indicated in children with suspected BrS, in children with a first-degree family member with definite or possible BrS according to the Shanghai criteria with a SCN5A variant and in paediatric SCN5A variant carriers. In-hospital rhythm monitoring during fever is indicated in patients with an existing type 1 ECG pattern and in those who develop such a pattern. Genetic testing should be restricted to SCN5A. Children with BrS and children who carry an SCN5A variant should avoid medication listed at www.brugadadrugs.org and fever should be suppressed. Ventricular arrhythmias or electrical storms should be treated with isoproterenol infusion.
摘要：
Brugada综合征（BrS）是一种罕见的遗传性心律失常综合征。受影响的儿童可能会出现危及生命的症状，主要是在发烧期间。儿童中SCN5A变异携带者的百分比高于成人。对于有（有）BrS家族史的儿童，目前的诊断和后续政策因中心而异。这里,我们在现有文献和专家意见的基础上提出了一项共识声明,以规范所有BrS儿童和荷兰BrS家庭儿童的治疗方法.总之,在自发性1型心电图（ECG）模式或上海评分≥3.5（包括≥1次ECG发现）的患者中诊断为BrS。钠通道阻断药物攻击试验只应在青春期后进行，只有少数例外。怀疑有BrS的儿童出现发热心电图，根据上海标准，具有明确或可能的BrS的一级家庭成员的儿童具有SCN5A变异体，以及儿科SCN5A变异体携带者。在存在1型ECG模式的患者和出现这种模式的患者中，需要在发烧期间进行院内节律监测。基因检测应仅限于SCN5A。患有BrS的儿童和携带SCN5A变体的儿童应避免使用www上列出的药物。brugadadrugs.org和发烧应该被抑制。室性心律失常或电风暴应通过异丙肾上腺素输注治疗。
