Brugada Syndrome: Diagnosis and Management

Brugada syndrome associated with high incidence of sudden death in young and otherwise healthy adults in many parts of the world. The Brugada syndrome which is characterized by an ST-segment elevation in the right precordial ECG leads, is a familial disease that displays an autosomal dominant mode o...

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Main Authors: Reza Octavianus, Yoga Yuniadi
Format: Article
Language:English
Published: Indonesian Heart Association 2013-06-01
Series:Majalah Kardiologi Indonesia
Subjects:
Online Access:http://ijconline.id/index.php/ijc/article/view/62
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author Reza Octavianus
Yoga Yuniadi
author_facet Reza Octavianus
Yoga Yuniadi
author_sort Reza Octavianus
collection DOAJ
description Brugada syndrome associated with high incidence of sudden death in young and otherwise healthy adults in many parts of the world. The Brugada syndrome which is characterized by an ST-segment elevation in the right precordial ECG leads, is a familial disease that displays an autosomal dominant mode o? transmission with incomplete penetrance. The first and only gene to be linked to Brugada syndrome is SCN5A, the gene that encodes for the subunit of the cardiac sodium channel gene. More than 80 mutations in SCN5A have been linked to the syndrome. Based on ECG characteristics the syndrome divided into three types. Brugada syndrome is definitively diagnosed when a type 1 ST-segment elevation is observed in >1 right precordial lead (V1 to V3) in the presence or absence of a sodium channel blocking agent, and in conjunction with one of the following: documented ventricular fibrillation (VF), polymorphic ventricular tachycardia (VT), a family history of sudden cardiac death at < 45 years old, coved-type ECGs in family members, inducibility of VT with programmed electrical stimulation, syncope, or nocturnal agonal respiration. Currently, an implantable cardioverter defibrilla?or (ICD) is the only proven effective treatment for the disease.
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spelling doaj.art-479446a1c044458ea24ce437c481b2ea2022-12-22T02:29:06ZengIndonesian Heart AssociationMajalah Kardiologi Indonesia0126-37732620-47622013-06-0133210.30701/ijc.v33i2.62Brugada Syndrome: Diagnosis and ManagementReza Octavianus0Yoga Yuniadi1Department of Cardiology and Vascular Medicine, Faculty of Medicine, University of Indonesia, and National Cardiovascular Center Harapan Kita JakartaDepartment of Cardiology and Vascular Medicine, Faculty of Medicine, University of Indonesia, and National Cardiovascular Center Harapan Kita JakartaBrugada syndrome associated with high incidence of sudden death in young and otherwise healthy adults in many parts of the world. The Brugada syndrome which is characterized by an ST-segment elevation in the right precordial ECG leads, is a familial disease that displays an autosomal dominant mode o? transmission with incomplete penetrance. The first and only gene to be linked to Brugada syndrome is SCN5A, the gene that encodes for the subunit of the cardiac sodium channel gene. More than 80 mutations in SCN5A have been linked to the syndrome. Based on ECG characteristics the syndrome divided into three types. Brugada syndrome is definitively diagnosed when a type 1 ST-segment elevation is observed in >1 right precordial lead (V1 to V3) in the presence or absence of a sodium channel blocking agent, and in conjunction with one of the following: documented ventricular fibrillation (VF), polymorphic ventricular tachycardia (VT), a family history of sudden cardiac death at < 45 years old, coved-type ECGs in family members, inducibility of VT with programmed electrical stimulation, syncope, or nocturnal agonal respiration. Currently, an implantable cardioverter defibrilla?or (ICD) is the only proven effective treatment for the disease.http://ijconline.id/index.php/ijc/article/view/62Brugada syndromereview
spellingShingle Reza Octavianus
Yoga Yuniadi
Brugada Syndrome: Diagnosis and Management
Majalah Kardiologi Indonesia
Brugada syndrome
review
title Brugada Syndrome: Diagnosis and Management
title_full Brugada Syndrome: Diagnosis and Management
title_fullStr Brugada Syndrome: Diagnosis and Management
title_full_unstemmed Brugada Syndrome: Diagnosis and Management
title_short Brugada Syndrome: Diagnosis and Management
title_sort brugada syndrome diagnosis and management
topic Brugada syndrome
review
url http://ijconline.id/index.php/ijc/article/view/62
work_keys_str_mv AT rezaoctavianus brugadasyndromediagnosisandmanagement
AT yogayuniadi brugadasyndromediagnosisandmanagement