Marcus Frank I
Sarver Heart Center, Department of Medicine, Section of Cardiology, University of Arizona, Tucson, AZ, 85724-5037, USA.
J Electrocardiol. 2005 Oct;38(4 Suppl):60-3. doi: 10.1016/j.jelectrocard.2005.06.007.
Investigation of the etiology and pathogeneses of the genetic cardiac channelopathies has provided important insight into the mechanisms of ventricular arrhythmias. Some of these diseases are rare, such as the short QT-interval syndrome, but others such as the Brugada syndrome are the most common cause of unexplained sudden cardiac death in young men in Southeast Asia and Japan. Knowledge of the specific ion-channel defect causing the disease or syndrome is leading to pharmacological approaches that attempt to correct the ion transfer defects in channelopathies. The most clinically important challenge is to recognize which patients with the Brugada electrocardiogram who do not have a positive family history of this condition and do not have a history of syncope or aborted sudden death are at risk for arrhythmic death.
对遗传性心脏离子通道病的病因和发病机制的研究，为室性心律失常的机制提供了重要的见解。其中一些疾病很罕见，如短QT间期综合征，但其他疾病，如Brugada综合征，是东南亚和日本年轻男性不明原因心脏性猝死的最常见原因。了解导致疾病或综合征的特定离子通道缺陷，正促使人们采取药物治疗方法，试图纠正离子通道病中的离子转运缺陷。临床上最重要的挑战是识别哪些没有Brugada综合征家族史、没有晕厥或心脏骤停病史的Brugada心电图患者有发生心律失常性死亡的风险。
