A 41-year-old man with a history of schizophrenia(精神分裂症) had a sudden cardiac arrest while touring a local fire department. The initial rhythm(节律) noted by emergency medical personnel was ventricular fibrillation(室颤). Three rounds of cardiopulmonary resuscitation were administered, with three defibrillations(除颤). The patient had return of spontaneous circulation and was transported to the hospital, where he was admitted to the critical care unit. Family members report that he has no known coronary artery disease risk factors and exercises(锻炼) regularly without chest pain. He does not take any medications for his schizophrenia, and family members do not believe that he uses any illicit substances. Physical examination is unremarkable. His first set of cardiac enzymes reveal a creatinine kinase level of 500 U/L, creatinine kinase MB 19 U/L, and troponin I 1.8 μg/mL. His ECG on arrival is shown below.
Which of the following is the most appropriate next step in management?
A. Implantable(可植入式) automatic defibrillator
B. Thrombolysis 溶栓
C. Procainamide 普鲁卡因
D. Beta-blocker
E. Amiodarone 胺碘酮
答案解析:
该患者的临床病史和ECG结果是典型的Brugada综合征。Brugada综合征患者最常见的表现是晕厥、室颤诱导的心脏骤停和心源性死亡。ECG结果遵循Brugada模式,包括假性右束支传导阻滞和V1-V3导联持续性ST段抬高。曾有孤立病例出现涉及下壁ECG导联的类似结果。以前,在实践中描述和使用了三种不同的ST段抬高模式;随后的共识是有两种不同的模式:在1型中,大于2 mm的抬高ST段随着向上凸起下降到倒置的T波。这被称为coved-type Brugada模式。在2型中,ST段具有“马鞍型”ST-T波构型,其中抬高的ST段下降至基线,然后再次上升至直立或双相T波。
该患者的ECG显示1型Brugada模式。诊断时的平均年龄为41岁,由于未知原因,精神分裂症患者比一般人群更可能出现Brugada模式ECG。精神分裂症药物似乎没有显著影响,Brugada模式和这种相关性的临床意义尚不确定。由于心脏钠通道突变,该综合征为常染色体显性遗传。在15%-30%的患者中发现了α亚基基因SCN5A的突变。
应考虑使用植入式除颤器来预防室颤复发和猝死。溶栓不适用于Brugada综合征患者。基线ECG正常的心脏骤停后接受1a型抗心律失常药(如普鲁卡因胺)治疗的患者可能引起与Brugada模式相似的ECG结果。β受体阻滞剂和胺碘酮对该综合征无帮助,应避免使用。
正确答案:A
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