Long QT syndrome: from channels to cardiac arrhythmias
Arthur J. Moss, Robert S. Kass
Arthur J. Moss, Robert S. Kass
Published August 1, 2005
Citation Information: J Clin Invest. 2005;115(8):2018-2024. https://doi.org/10.1172/JCI25537.
View: Text | PDF
Review Series

Long QT syndrome: from channels to cardiac arrhythmias

Abstract

Long QT syndrome, a rare genetic disorder associated with life-threatening arrhythmias, has provided a wealth of information about fundamental mechanisms underlying human cardiac electrophysiology that has come about because of truly collaborative interactions between clinical and basic scientists. Our understanding of the mechanisms that control the critical plateau and repolarization phases of the human ventricular action potential has been raised to new levels through these studies, which have clarified the manner in which both potassium and sodium channels regulate this critical period of electrical activity.

Authors

Arthur J. Moss, Robert S. Kass

×

Figure 1

Options: View larger image (or click on image) Download as PowerPoint
(A) An illustrative example of a single cardiac cycle detected as spatia...
(A) An illustrative example of a single cardiac cycle detected as spatial and temporal electrical gradients on the ECG. The P wave is generated by the spread of excitation through the atria. The QRS complex represents ventricular activation and is followed by the T wave, which reflects ventricular repolarization gradients. (B) Schematic representation of the KCNH2 (HERG) potassium channel α subunit, involving the N-terminal part (NH3+), 6 membrane-spanning segments with the pore region located from segment S5 to segment S6, and the C-terminal portion (COO–). Mutation locations are indicated by blue dots. Fourteen different mutations were located in 13 locations within the pore region. Reproduced with permission from Circulation (28).