Cellular basis for long QT, transmural dispersion of repolarization, and torsade de pointes in the long QT syndrome

Journal of Electrocardiology

Volumn 32, Issue SUPPL., 1999, Pages 177-184

  Shimizu, Wataru ^a   Antzelevitch, Charles ^b  

^a NATIONAL CEREBRAL AND CARDIOVASCULAR CENTER   (Japan)

^b MASONIC MEDICAL RESEARCH LABORATORY   (United States)

Author keywords

Arrhythmias; HERG; Heterogeneity; KCNE1; KvLQT1; Long QT syndrome; M cell; SCN5A

Indexed keywords

ION CHANNEL; ISOPRENALINE; MEXILETINE; PROPRANOLOL;

CELL HETEROGENEITY; CONFERENCE PAPER; DOG; ELECTROSTIMULATION; GENE MUTATION; HEART REPOLARIZATION; LONG QT SYNDROME; MEMBRANE POTENTIAL; PHENOTYPE; PRIORITY JOURNAL; T WAVE; TORSADE DES POINTES;

EID: 0033368409     PISSN: 00220736     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0022-0736(99)90077-8     Document Type: Conference Paper

References (43)

1. Schwartz PJ, Periti M, Malliani A: The long QT syndrome. Am Heart J 89:378, 1975
2. Moss AJ, Schwarte PJ, Crampton RS, et al: Hereditable malignant arrhythmias: the long QT syndrome: a prospective international study. Circulation 71:17, 1985
3. Shimizu W, Ohe T, Kurita T, et al: Early afterdepolarizations induced by isoproterenol in patients with congenital long QT syndrome. Circulation 84:1915, 1991
4. Schwartz PJ, Moss AJ, Vincent GM, Crampton RS: Diagnostic criteria for the long QT syndrome: an update. Circulation 88:782, 1993
5. Roden DM, Lazzara R, Rosen MR, et al: Multiple mechanisms in the long-QT syndrome: current knowledge, gaps, and future directions. Circulation 94:1996, 1996
6. Wang Q, Shen J, Splawski I, et al: SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell 80:805, 1995
7. Kr potassium channel. Cell 81:299, 1995
8. Curran ME, Splawski I, Timothy KW, et al: A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell 80:795, 1995
9. Schott JJ, Charpentier F, Peltier S, et al: Mapping of a gene for long QT syndrome to chromosome 4q25-27. Am J Hum Genet 57:1114, 1995
10. Wang Q, Curran ME, Splawski I, et al: Positional cloning of a novel potassium channel gene: KvLQT1 mutations cause cardiac arrhythmias. Nature Genet 12:17, 1996
11. Ks potassium channel. Nature 384:80, 1996
12. Ks cardiac potassium current. Nature 384:78, 1996
13. Ks function. Nat Genet 17:338, 1997
14. Splawski I, Timothy KW, Vincent GM, et al: Molecular basis of the long-QT syndrome associated with deafness. N Engl J Med 336:1562, 1997
15. Neyroud N, Tesson F, Leibovici M, et al: A novel mutation in the potassium channel gene KvLQT1 caused the Jervell and Lange-Nielsen cardioauditory syndrome. Nat Genet 15:186, 1997
16. Shimizu W, Antzelevitch C: Sodium channel block with mexiletine is effective in reducing dispersion of repolarization and preventing torsade de pointes in LQT2 and LQT3 models of the long-QT syndrome. Circulation 96:2038, 1997
17. Shimizu W, Antzelevitch C: Cellular basis for the electrocardiographic features of the LQT1 form of the long QT syndrome: effects of β-adrenergic agonists, antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes. Circulation 98:2314, 1998
18. Yan GX, Shimizu W, Antzelevitch C: The characteristics and distribution of M cells in arterially-perfused canine left ventricular wedge preparations. Circulation 98:1921, 1998
19. Yan GX, Antzelevitch C: Cellular basis for the normal T wave and the electrocardiographic manifestations of the long QT syndrome. Circulation 98:1928, 1998
20. Shimizu W, Antzelevitch C: Cellular and ionic basis for T wave alternans under long QT syndrome conditions. Circulation 99:1499, 1999
21. Shimizu W, Antzelevitch C: Sodium pentobarbital reduces transmural dispersion of rcpolarization and prevents torsade de pointes in models of acquired and congenital long QT syndrome. J Cardiovasc Electrophysiol 10:154, 1999
22. Moss AJ, Zareba W, Benhorin J, et al: ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation 92:2929, 1995
23. Sicouri S, Antzelevitch C: A subpopulation of cells with unique electrophysiological properties in the deep subepicardium of the canine ventricle. The M cell. Circ Res 68:1729, 1991
24. Antzelevitch C, Sicouri S, Litovsky SH, et al: Heterogeneity within the ventricular wall: electrophysiology and pharmacology of epicardial, endocardial and M cells. Circ Res 69:1427, 1991
25. Antzelevitch C, Sicouri S: Clinical relevance of cardiac arrhythmias generated by afterdepolarizations: the role of M cells in the generation of U waves, triggered activity and torsade de pointes. J Am Coll Cardiol 23:259, 1994
26. Antzelevitch C, Shimizu W, Yan GX et al: The M cell: its contribution to the ECG and to normal and abnormal function of the heart. J Cardiovasc Electrophysiol 10:1124, 1999
27. Antzelevitch C, Yan GX, Shimizu W, Burashnikov A: Electrical heterogeneity as the basis for the ECG and function of the heart in health and disease. In Zipes DP, Jalife J (eds): Cardiac electrophysiology: from cell to bedside, 3rd ed. WB Saunders, Philadelphia, 1999 (in press)
28. Shimizu W, Ohe T, Kurita T, et al: Effects of verapamil and propranolol on early afterdepolarizations and ventricular arrhythmias induced by epinephrine in congenital long QT syndrome. J Am Coll Cardiol 26:1299, 1995
29. + channel opener in the LQT1 form of congenital long QT syndrome. Circulation 97:1581, 1998
30. Vos MA, Verduyn SC, Gorgels APM, et al: Reproducible induction of early afterdepolarizations and torsade de pointes arrhythmias by D-sotalol and pacing in the dog with chronic atrioventricular block. Circulation 91:864, 1995
31. El-Sherif N, Caref EB, Yin H, Restivo M: The electrophysiological mechanism of ventricular arrhythmias in the long QT syndrome: tridimensional mapping of activation and recovery patterns. Circ Res 79:474, 1996
32. El-Sherif N, Chinushi M, Caref EB, Restivo M: Electrophysiological mechanism of the characteristic electrocardiographic morphology of torsade de pointes tachyarrhythmias in the long-QT syndrome. Detailed analysis of ventricular tridimensional activation patterns. Circulation 96:4392, 1997
33. Moss AJ, Liu JE, Gottlieb S, et al: Efficacy of permanent pacing in the management of high-risk patients with long QT syndrome. Circulation 84:1524, 1991
34. + channel blockade and to increases in heart rate: implications for gene-specific therapy. Circulation 92:3381, 1995
35. + channel blockade, β-adrenergic stimulation, and rapid pacing in a cellular model mimicking the SCN5A and HERG defects present in the long-QT syndrome. Circ Res 78:1009, 1996
36. Hirao H, Shimizu W, Kurita T, et al: Frequency-dependent electrophysiologic properties of ventricular repolarization in patients with congenital long QT syndrome. J Am Coll Cardiol 28:1269, 1996
37. Viskin S, Alia SR, Barron HL, et al: Mode of onset of torsade de pointes in congenital long QT syndrome. J Am Coll Cardiol 28:1262, 1996
38. Compton SJ, Lux RL, Ramsey MR, et al: Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium. Circulation 94:1018, 1996
39. Moss AJ, Schwartz PJ, Crampton RS, et al: The long QT syndrome: prospective longitudinal study of 328 families. Circulation 84:1136, 1991
40. Vincent GM, Fox J, Zhang L, Timothy KW: Beta-blockers markedly reduce risk and syncope in KvLQT1 long QT patients (abstract). Circulation 94:1-204, 1996
41. Wilde AAM, Jongbloed RJE, Doevendans PA, et al: Auditory stimuli as a trigger for arrhythmic events differentiate HERG-related (LQT2) patients from KvLQT1-related patients (LQT1). J Am Coll Cardiol 33:327, 1999
42. Shimizu W, Antzelevitch C: Different effects of β-adrenergic agonists and antagonists in LQT1, LQT2 and LQT3 models of the long QT syndrome. J Am Cell Cardiol (in press)
43. Shimizu W, Antzelevitch C: Differential effects of a K+ channel opener in reducing dispersion of repolarization and preventing torsade de pointes in LQT1, LQT2 and LQT3 models of the long QT syndrome (abstract). PACE 21:II-846, 1998