Screening, diagnosis and follow-up of Brugada syndrome in children: a Dutch expert consensus statement.

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 EC

SUBMITTER: Peltenburg PJ 

PROVIDER: S-EPMC9554382 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature