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RESEARCH PRODUCT

Update on Brugada Syndrome 2019

Pedro BrugadaEgle CorradoGiampiero MagliaAntonio CurnisGiuseppe CoppolaDomenico OrienteAntonino Mignano

subject

medicine.medical_specialtymedicine.medical_treatment030204 cardiovascular system & hematologyRisk Assessmentsudden cardiac deathSudden cardiac deathElectrocardiography03 medical and health sciences0302 clinical medicineInternal medicinemedicineHumansST segmentBrugada syndromeIn patient030212 general & internal medicineCardiac disordersBrugada syndromeMedicine(all)business.industryGenetic heterogeneityGeneral Medicinemedicine.diseaseImplantable cardioverter-defibrillatorBrugada Syndrome.Defibrillators ImplantableCardiologyhigh incidenceCardiology and Cardiovascular MedicinebusinessRisk assessment

description

Brugada syndrome (BrS) was first described in 1992 as an aberrant pattern of ST segment elevation in right precordial leads with a high incidence of sudden cardiac death (SCD) in patients with structurally normal heart. It represents 4% ∼ 12% of all SCD and 20% of SCD in patients with structurally normal heart. The extremely wide genetic heterogeneity of BrS and other inherited cardiac disorders makes this new area of genetic arrhytmology a fascinating one. This review shows the state of art in diagnosis, management, and treatment of BrS focusing all the aspects regarding genetics and Preimplant Genetic Diagnosis (PGD) of embryos, overlapping syndromes, risk stratification, familial screening, and future perspectives. Moreover the review analyzes key points like electrocardiogram (ECG) criteria, the role of electrophysiological study (the role of ventricular programmed stimulation and the need of universal accepted protocol) and the importance of a correct risk stratification to clarify when implantable cardioverter defibrillator or a close follow-up is needed. In recent years, cardiovascular studies have been focused on personalized risk assessment and to determine the most optimal therapy for an individual. The BrS syndrome has also benefited of these advances although there remain several key points to be elucidated. We will review the present knowledge, progress made, and future research directions on BrS.

yearjournalcountryeditionlanguage
2021-03-01Current Problems in Cardiology
https://doi.org/10.1016/j.cpcardiol.2019.100454