Franco Emiliana, Dias Andre, Teresa Daniele, Hebert Kathy
Danbury Hospital, Internal Medicine, Danbury, Connecticut.
Ann Noninvasive Electrocardiol. 2014 Mar;19(2):198-202. doi: 10.1111/anec.12086. Epub 2013 Sep 9.
Brugada Syndrome (BrS) is a cardiac disorder characterized by incomplete right bundle-branch block and ST elevations in the anterior precordial leads especially V1 -V3 , associated with an increased risk for sudden cardiac death (SCD) in young adults. Our case describes a patient with family history of sudden infant death syndrome (SIDS) who presented with a Brugada pattern unmasked by severe hyperkalemia and diabetic ketoacidosis. Several studies have concluded there may be a genetic link among SIDS, SDC, and BrS resulting from mutations in cardiac ion channel-related genes. Recognizing SIDS as part of the diagnostic criteria for BrS would help us identifying a significant number of families susceptible to develop SCD (as well as SIDS).
Brugada综合征(BrS)是一种心脏疾病,其特征为不完全性右束支传导阻滞以及胸前导联尤其是V1 - V3导联ST段抬高,与年轻成年人心脏性猝死(SCD)风险增加相关。我们的病例描述了一名有婴儿猝死综合征(SIDS)家族史的患者,该患者出现了由严重高钾血症和糖尿病酮症酸中毒所揭示的Brugada波型。多项研究得出结论,由于心脏离子通道相关基因突变,SIDS、SDC和BrS之间可能存在遗传联系。将SIDS纳入BrS的诊断标准将有助于我们识别大量易发生SCD(以及SIDS)的家庭。