Peculiarities of clinical presentation and diagnosis of Brugada syndrome in children and adolescents

Sudden cardiac death (SCD) in pediatric population occurs with an incidence of 0.7-6.4 cases per 100,000 population. The leading mechanism of VS in this case is malignant arrhythmias. One of the conditions leading to their development is Brugada syndrome. Brugada syndrome is a genetically and phenotypically heterogeneous hereditary disease characterized by a malignant clinical and electrographic arrhythmic syndrome that manifests as spontaneous or provoked, persistent or recurrent ST segment elevation of a vaulted configuration in the right thoracic leads (Brugada pattern type I), right bundle branch block on ECG and clinically in the form of recurrent syncope or episodes of sudden cardiac death due to malignant ventricular arrhythmias (polymorphic ventricular tachycardia or ventricular fibrillation). Although significant progress has been made in understanding the pathophysiologic processes of the disease and diagnostic criteria and risk stratification have been proposed for adult patients, in children the diagnosis of this disease is difficult because of the high frequency of asymptomatic course and imperfect interpretation of electrocardiogram recordings. Thus, the aim of this review is to compare the effectiveness of the applied diagnostic methods and to identify additional opportunities for diagnosing SB in children and adolescents. The results of 19 original studies, reviews of current literature and descriptions of rare clinical cases published in the last 5 years were analyzed. Thus, according to a number of studies, the most frequent manifestations of this syndrome in children are hereditary anamnesis and incidental ECG findings, syncopal states and arrhythmias at this age are registered much less frequently. The main conclusion obtained in the course of the literature review is to expand the possibilities of diagnostics of Brugada syndrome when recording ECG during the action of provoking electrocardiographic changes, such as febrile temperature, drugs and periods of high parasympathetic tone, which determines a higher probability of diagnosing this syndrome in patients of infectious diseases departments if ECG is recorded during fever and in patients who had daily ECG monitoring by Holter. It is also worth noting that an important aspect in risk stratification for children is genetic research, as a higher frequency of malignant arrhythmias and their recurrences among patients with a confirmed mutation in the SCN5A gene than in adults was revealed.