Patients with Brugada syndrome (BrS) who have genetic mutations of SCN5A tend to develop symptoms at a younger age, and have more pronounced electrophysiological defects (e.g., higher rate of spontaneous type 1 ECG pattern, significant conduction or repolarisation abnormalities, increased atrial vulnerability) and a more severe prognosis.[92]Chen C, Tan Z, Zhu W, et al. Brugada syndrome with SCN5A mutations exhibits more pronounced electrophysiological defects and more severe prognosis: A meta-analysis. Clin Genet. 2020 Jan;97(1):198-208.
http://www.ncbi.nlm.nih.gov/pubmed/30963536?tool=bestpractice.com
Age ≥55 at diagnosis is associated with a more benign prognosis, with no increased mortality compared with the general population.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405.
https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
Established features that are associated with the greatest risk of serious arrhythmic events in patients with BrS are:[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405.
https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
[40]Gonzalez Corcia MC, Sieira J, Sarkozy A, et al. Brugada syndrome in the young: an assessment of risk factors predicting future events. Europace. 2017 Nov 1;19(11):1864-73.
https://academic.oup.com/europace/article/19/11/1864/2194460?login=false
http://www.ncbi.nlm.nih.gov/pubmed/27738063?tool=bestpractice.com
[86]Andorin A, Behr ER, Denjoy I, et al. Impact of clinical and genetic findings on the management of young patients with Brugada syndrome. Heart Rhythm. 2016 Jun;13(6):1274-82.
http://www.ncbi.nlm.nih.gov/pubmed/26921764?tool=bestpractice.com
[87]Priori SG, Gasparini M, Napolitano C, et al. Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry. J Am Coll Cardiol. 2012;59(1):37-45.
https://www.sciencedirect.com/science/article/pii/S073510971104530X?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/22192666?tool=bestpractice.com
[88]Priori SG, Napolitano C, Gasparini M, et al. Natural history of Brugada syndrome: insights for risk stratification and management. Circulation. 2002;105(11):1342-7.
https://www.ahajournals.org/doi/10.1161/hc1102.105288?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
http://www.ncbi.nlm.nih.gov/pubmed/11901046?tool=bestpractice.com
Resuscitated cardiac arrest
History of cardiogenic syncope
Spontaneous type 1 Brugada pattern on ECG.