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心脏电疾病的分类极端复杂,许多方面甚至相互矛盾。AHA在致心律失常心肌病的分类法中,建议把离子通道病合并到原发性心肌病中。2007年11月,美国国立心肺与血管研究所和罕见疾病办公室在Circulation杂志发表了关于由基因突变影响离子通道功能所致原发性心肌病的诊断、表型、分子机制和治疗手段的专家共识报告,针对这组日益复杂化的原发性心脏膜通道疾病提出了一个重要框架。现就其中的重点与新进展做一综述。内容包括:(1)Na+通道病;(2)K+通道突变引起的心律失常;(3)由于其它遗传性致心律失常机制引起的心律失常;(4)未来诊断和治疗的建议。
The classification of cardiac electrical disorders is extremely complex and in many respects even contradictory. In the classification of arrhythmogenic cardiomyopathy by AHA, it is advisable to incorporate ion channel disease into primary cardiomyopathy. In November 2007, the National Institute of Cardiovascular and Vascular Research and the Rare Disease Office published an expert consensus in Circulation on the diagnosis, phenotype, molecular mechanism and treatment of primary cardiomyopathy due to ion channel function due to gene mutations The report presents an important framework for this group of increasingly complex primary cardiac membrane disease. Now on the focus and new progress to be reviewed. (1) Na + channel disease; (2) arrhythmia caused by K + channel mutation; (3) arrhythmia due to other genetic induced arrhythmia; (4) future diagnosis and treatment recommendations.