Present Status of Brugada Syndrome JACC State-of-the-Art Review

被引:266
作者
Brugada, Josep [1 ,2 ,3 ]
Campuzano, Oscar [3 ,4 ,5 ]
Arbelo, Elena [1 ,3 ]
Sarquella-Brugada, Georgia [2 ,5 ]
Brugada, Ramon [3 ,4 ,5 ,6 ]
机构
[1] Univ Barcelona, Inst Clin Cardiovasc ICCV, Hosp Clin, Inst Invest Biomed August Pi & Sunyer IDIBAPS, C Villarroel 170,6-3, E-08036 Barcelona, Spain
[2] Univ Barcelona, Hosp St Joan de Deu, Arrhythmias Unit, Barcelona, Spain
[3] Ctr Invest Biomed Red Enfermedades Cardiovasc CIB, Madrid, Spain
[4] Univ Girona, Cardiovasc Genet Ctr, Inst Invest Biomed Girona IDIBGI, Girona, Spain
[5] Univ Girona, Sch Med, Med Sci Dept, Girona, Spain
[6] Hosp Josep Trueta, Cardiol Serv, Girona, Spain
关键词
arrhythmias; Brugada syndrome; genetics; sudden cardiac death; ST-SEGMENT ELEVATION; SUDDEN CARDIAC DEATH; BUNDLE-BRANCH BLOCK; IMPLANTABLE CARDIOVERTER-DEFIBRILLATOR; PROGRAMMED ELECTRICAL-STIMULATION; VENTRICULAR OUTFLOW TRACT; LONG-TERM PROGNOSIS; T-WAVE ALTERNANS; RISK STRATIFICATION; EARLY REPOLARIZATION;
D O I
10.1016/j.jacc.2018.06.037
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
The Brugada syndrome is an inherited disorder associated with risk of ventricular fibrillation and sudden cardiac death in a structurally normal heart. Diagnosis is based on a characteristic electrocardiographic pattern (coved type ST-segment elevation >= 2 mm followed by a negative T-wave in >= 1 of the right precordial leads V-1 to V-2), observed either spontaneously or during a sodium-channel blocker test. The prevalence varies among regions and ethnicities, affecting mostly males. The risk stratification and management of patients, principally asymptomatic, still remains challenging. The current main therapy is an implantable cardioverter-defibrillator, but radiofrequency catheter ablation has been recently reported as an effective new treatment. Since its first description in 1992, continuous achievements have expanded our understanding of the genetics basis and electrophysiological mechanisms underlying the disease. Currently, despite several genes identified, SCN5A has attracted most attention, and in approximately 30% of patients, a genetic variant may be implicated in causation after a comprehensive analysis. (C) 2018 by the American College of Cardiology Foundation.
引用
收藏
页码:1046 / 1059
页数:14
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