BRUGADA SYNDROME: NEWEST DATA ON RISK STRATIFICATION

Abstract

Abstract Background Brugada Syndrome (BrS) is a rare genetically based channelopathy responsible for malignant arrhythmias and sudden cardiac death (SCD) in young adults. Objectives The aims was to evaluate prognostic indicators useful for accurate arrhythmic risk stratification in BrS by studying genetic, clinical, anamnestic, instrumental parameters, and those derived from electrophysiological study (EPS) and intracavitary mapping. Materials and Methods 209 patients with Brugada type 1 were recruited between 01/01/2018 and 01/10/2023. Results In the first study, predictive factors related to high arrhythmic risk from the data obtained for the total population of 209 Brugada patients enrolled in our case series include a positive history of cardiogenic syncope (p value 0.000041), having had a resuscitated cardiac arrest (p value 0.00025), presenting positive EPS for inducibility of ventricular tachycardia (p value 0.00032), having a family history of sudden cardiac death (p value < 0.00001), and presenting positive cardiac mapping and MRI for pathological alterations (both with p value > 0.00001). From the data obtained for the subset of 80 Brugada patients with ICD in our case series, predictive factors related to high arrhythmic risk are different from the previously analyzed population: presenting spontaneous type 1 ECG (p value 0.0015), having had a resuscitated cardiac arrest (p value 0.014028), having a family history of sudden cardiac death (p value < 0.00001), and presenting positive mapping, genetics, and cardiac MRI for pathological alterations. Positive EPS and syncope in this subset do not appear to be risk factors, unlike the population of all 209 enrolled patients. Conclusions Multiparametric scores, including the mentioned markers, have been introduced in clinical practice for a better approach in selection. However, new markers are needed to improve risk stratification in BrS patients, especially those in the so–called "gray zone" with intermediate risk. In the future, it will be evaluated through experimental models and genotype–phenotype integration whether such electrical heterogeneity is also associated with specific genetic or structural abnormalities and may represent a new therapeutic target for etiological treatment in BrS patients. In conclusion, there is an urgent need to develop new methods for risk stratification in Brugada syndrome that are sufficiently sensitive, specific, reproducible, and easy to apply.