Brugada Syndrome: Defining the Risk in Asymptomatic Patients

Abstract

Since the first description of the Brugada syndrome (BS) in 1992, scientific progress in the understanding of this disease has been enormous; at the same time more and more individuals with the disease have been diagnosed. The profile of patients with BS has changed with more asymptomatic individuals and less expressive clinical features. Asymptomatic BS individuals are at lower arrhythmic risk than those presenting with syncope or sudden cardiac death (SCD). The event incidence rate is around 0.5 % per year; this figure is relevant due to the fact that individuals have a long life expectancy and are otherwise healthy. As a result of the risk of SCD, risk stratification is of utmost importance. As the implantation of a cardioverter defibrillator is the main treatment for those patients at higher risk, benefits and long-term potential risks have to be adequately considered. Some risk factors, such as spontaneous type 1 electrocardiogram (ECG) pattern, are widely accepted, whilst for others contradictory data are present. Furthermore, novel risk factors are now available that might help in the management of BS. The presence of a spontaneous type 1 ECG pattern, history of sinus node dysfunction and inducible ventricular arrhythmias during programmed electrical stimulation of the heart allow us to risk stratify these patients.

Brugada syndrome in a young patient with type 1 myotonic dystrophy requiring an implantable cardioverter defibrillator for primary prevention: a case report

“…In general, ventricular stimulation studies are not currently recommended by the guidelines in patients with drug-induced BS. However, Pedro Brugada's group have studied a large cohort of BS patients and advocate electrophysiologic testing which seems to have good predictive value especially when less aggressive protocols (up to two extrastimuli) are used 3 , 11 . In our patient who was not having spontaneous Type 1 BS this strategy and the subsequent ICD implantation proved to be life-saving.…”

Brugada Syndrome: Presentation and Management of the Atypical Patient in the Emergent Setting

“…ICD has the greatest efficacy in averting sudden cardiac death, but the decision to use the device depends on the patient's ability to tolerate it. 7 If a patient cannot tolerate an ICD, pharmacologic therapy then serves as a second-line treatment modality. Pharmaceuticals can be used in the acute management of an arrhythmic storm, to prevent arrhythmia in patients with an implanted ICD requiring multiple shocks, and in cases where ICD implantation is contraindicated or not feasible -as may be the case in children or upon patient refusal.…”

A Case of Brugada Pattern Associated with Adrenal Insufficiency

“…Spontaneous type 1 ECG, inducible ventricular arrhythmias during an electrophysiological study, and the presence of sinus node dysfunction can identify patients at a higher risk. Novel risk markers might also help in their management [ 1 ].…”

“…Brugada syndrome (BrS) is an inherited disease characterized by a coved-type ST-segment elevation in the right precordial leads (V1–V3) and an increased risk of sudden cardiac death (SCD) in the absence of a structural heart disease [ 1 ]. Common triggers for the Brugada pattern include exercise, fever, ischemia, and certain medications.…”