Brugada Syndrome: Focus for the General Pediatrician

Abstract

Brugada Syndrome is an "inherited" channelopathy characterized by a predisposition to syncope and sudden death. It typically presents in young adults but is also known to affect the pediatric population, even if the prevalence is low compared to the adult population. The diagnostic ECG pattern shows coved-type ST-segment elevation in the right precordial leads, occurring spontaneously or after provocative drug tests with IV administration of Class I antiarrhythmic drugs. However, the electrocardiographic findings may vary, and transient or concealed forms of the syndrome further complicate diagnosis, necessitating thorough evaluation and close clinical follow-up. The clinical presentation of Brugada Syndrome may range from asymptomatic individuals to patients who have experienced syncope or sudden cardiac arrest. The syndrome remains underdiagnosed due to its elusive symptoms and the absence of abnormal findings between episodes. Additionally, specific triggers such as fever, certain medications and alcohol consumption may unmask the electrocardiographic changes and provoke arrhythmias in susceptible individuals. Given its elusive nature, early diagnosis and risk stratification are crucial in identifying individuals who may benefit from an implantable cardioverter defibrillator, the mainstay of treatment for high-risk patients, or pharmacological interventions.

Keywords: Brugada pattern; Brugada syndrome; arrhythmia; pediatric population; sudden cardiac death.

Figure 1

The different morphologies of the Brugada pattern [16].

Figure 2

(Left) Standard-lead electrocardiogram positions. (Right) High V1–V2 position. Modified from Krahn A. et al., “Brugada Syndrome” [1].

Figure 3

Modified from Andorin et al., “Impact of clinical and genetic findings on managing young patients with Brugada syndrome” [28].