Levin Charlotte Glosli, Khalili Payam, Floderer Edit
Centralsjukhuset i Karlstad - Medicinkliniken Karlstad, Sweden Centralsjukhuset i Karlstad - Medicinkliniken Karlstad, Sweden.
Centralsjukhuset i Karlstad - Hjärt- och akutmedicinkliniken Karlstad, Sweden Centralsjukhuset i Karlstad - Hjärt- och akutmedicinkliniken Karlstad, Sweden.
Lakartidningen. 2015 Nov 24;112:DRFP.
Brugada syndrome is a rare hereditary condition comprising electrocardiographic findings and an increased risk of sudden death due to ventricular fibrillation. The transmission is autosomal dominant with incomplete penetrance, mainly affecting males. The clinical manifestations include syncope, sudden cardiac death, nocturnal agonal breathing, documented ventricular tachycardia/fibrillation, and inducibility of arrhythmias during electrophysiologial study. The ECG should typically have an appearance of a right bundle branch block with a coved ST-segment elevation ≥ 2 mm, followed by a negative T-wave, in at least one right-sided lead (V1-V2). Two cases of Brugada syndrome are hereby presented, both of whom received the definitive treatment - ICD.
布加综合征是一种罕见的遗传性疾病，其特征包括心电图表现以及因室颤导致的猝死风险增加。其遗传方式为常染色体显性遗传且具有不完全外显率，主要影响男性。临床表现包括晕厥、心源性猝死、夜间濒死呼吸、记录到的室性心动过速/室颤以及在电生理研究中可诱发心律失常。心电图通常表现为至少一个右侧导联（V1 - V2）出现右束支传导阻滞伴穹窿型ST段抬高≥2毫米，随后是倒置的T波。本文报告两例布加综合征病例，二者均接受了确定性治疗——植入式心律转复除颤器（ICD）。
