Antzelevitch C, Oliva A
Masonic Medical Research Laboratory, Utica, NY 3501-1787, USA.
J Intern Med. 2006 Jan;259(1):48-58. doi: 10.1111/j.1365-2796.2005.01587.x.
This review examines the hypothesis that amplification of spatial dispersion of repolarization in the form of transmural dispersion of repolarization (TDR) underlies the development of life-threatening ventricular arrhythmias associated with inherited ion channelopathies including the long QT, short QT and Brugada syndromes as well as catecholaminergic polymorphic ventricular tachycardia. In the long QT syndrome, amplification of TDR is often secondary to preferential prolongation of the action potential duration (APD) of M cells, whereas in the Brugada syndrome, it is thought to be because of selective abbreviation of the APD of right ventricular epicardium. Preferential abbreviation of APD of either endocardium or epicardium appears to be responsible for amplification of TDR in the short QT syndrome. In catecholaminergic polymorphic VT, the reversal of the direction of activation of the ventricular wall is responsible for the increase in TDR. In conclusion, the long QT, short QT, Brugada and catecholaminergic VT syndromes are pathologies with very different phenotypes and aetiologies, but which share a common final pathway in causing sudden death.
复极空间离散以跨壁复极离散（TDR）的形式放大，是包括长QT综合征、短QT综合征、Brugada综合征以及儿茶酚胺能多形性室性心动过速在内的与遗传性离子通道病相关的危及生命的室性心律失常发生发展的基础。在长QT综合征中，TDR的放大通常继发于M细胞动作电位时程（APD）的优先延长，而在Brugada综合征中，其被认为是由于右心室心外膜APD的选择性缩短。心内膜或心外膜APD的优先缩短似乎是短QT综合征中TDR放大的原因。在儿茶酚胺能多形性室性心动过速中，心室壁激活方向的逆转是TDR增加的原因。总之，长QT综合征、短QT综合征、Brugada综合征和儿茶酚胺能室性心动过速综合征是具有非常不同表型和病因的疾病，但在导致猝死方面有共同的最终途径。
