Zitron E, Scholz E P, Kiesecker C, Pirot M, Kathöfer S, Thomas D, Kiehn J, Katus H A, Becker R, Karle C A
AG Molekulare Elektrophysiologie, Medizinische Universitätsklinik Heidelberg, Innere Medizin III, 69120 Heidelberg.
Herzschrittmacherther Elektrophysiol. 2005 Dec;16(4):229-38. doi: 10.1007/s00399-005-0490-9.
The last decade has seen rapid progress in our understanding of the molecular basis of arrhythmias, particularly concerning hereditary arrhythmia syndromes. This has led to significant improvement regarding differentiation, risk stratification and therapy in these patients and their families. However, there is mounting evidence that the knowledge obtained by studying these rare monogenic disorders will also enable us to dissect the molecular mechanisms underlying polygenetic and multi-factorial arrhythmias that are by far more common in clinical practice. The goal of this review is to give a brief overview of current knowledge on the molecular basis of primary electrical heart diseases. A focus is on the long QT syndrome.
在过去十年中,我们对心律失常分子基础的理解取得了迅速进展,尤其是在遗传性心律失常综合征方面。这使得这些患者及其家族在鉴别诊断、风险分层和治疗方面有了显著改善。然而,越来越多的证据表明,通过研究这些罕见的单基因疾病所获得的知识,也将使我们能够剖析多基因和多因素心律失常的分子机制,而这些心律失常在临床实践中更为常见。本综述的目的是简要概述原发性心脏电疾病分子基础的当前知识。重点是长QT综合征。