Inherited long QT syndromes: A paradigm for understanding arrhythmogenesis

Journal of Cardiovascular Electrophysiology

Volumn 10, Issue 12, 1999, Pages 1664-1683

  Roden, Dan M ^a   Spooner, Peter M ^b  

^a VANDERBILT UNIVERSITY   (United States)

^b NATIONAL HEART LUNG AND BLOOD INSTITUTE   (United States)

Author keywords

Cardiac arrhythmias; Genetic diseases; Ion channels; Long QT syndrome; Sudden cardiac death

Indexed keywords

CALCIUM; ION CHANNEL;

ARRHYTHMOGENESIS; CALCIUM CELL LEVEL; EMOTION; EXERCISE; GENE MUTATION; GENETIC SCREENING; HEART ARRHYTHMIA; HEART ELECTROPHYSIOLOGY; HEART FAILURE; HEART HYPERTROPHY; HEART INFARCTION; HEART REPOLARIZATION; LONG QT SYNDROME; PRIORITY JOURNAL; QT INTERVAL; REST; REVIEW; SLEEP; SUDDEN DEATH; SUDDEN INFANT DEATH SYNDROME; SYNCOPE;

EID: 0033378863     PISSN: 10453873     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1540-8167.1999.tb00231.x     Document Type: Review

References (185)

1. 1. Schwartz PJ, Stramba-Badiale M, Segantini A, et al: Prolongation of the QT interval and the sudden infant death syndrome. N Engl J Med 1998;338:1709-1714.
2. 2. Roden DM, Lazzara R, Rosen MR, et al, The SADS Foundation Task Force on LQTS: Multiple mechanisms in the long QT syndrome: Current knowledge, gaps, and future directions. Circulation 1996;94:1996-2012.
3. 3. Kass RS, Davies MP: The roles of ion channels in an inherited heart disease: Molecular genetics of the long QT syndrome. Cardiovasc Res 1996;32:443-454.
4. 4. Moss AJ: Clinical management of patients with the long QT syndrome: Drugs, devices, and gene-specific therapy. PACE 1997;20:2058-2060.
5. 5. Ackerman MJ: The long QT syndrome: Ion channel diseases of the heart. Mayo Clin Proc 1998;73:250-269.
6. 6. Wang Q, Chen Q, Towbin JA: Genetics, molecular mechanisms and management of long QT syndrome. Ann Med 1998;30:58-65.
7. 7. Keating MT: The long QT syndrome. A review of recent molecular genetic and physiologic discoveries. Medicine 1996;75:1-5.
8. 8. Schwartz PJ, Priori SG, Napolitano C: Long QT syndrome. In Zipes DP, Jalife J, eds: Cardiac Electrophysiology: From Cell to Bedside. WB Saunders, Philadelphia, 1999, pp. 788-810.
9. 9. Keating M, Atkinson D, Dunn C, et al: Linkage of a cardiac arrhythmia, the long QT syndrome, and the Harvey ras-1 gene. Science 1991;252:704-706.
10. 10. Wang Q, Curran ME, Splawski I, et al: Positional cloning of a novel potassium channel gene: KVLQTI mutations cause cardiac arrhythmias. Nat Genet 1996; 12:17-23.
11. 11. Takumi T, Ohkubo H, Nakanishi S: Cloning of a membrane protein that induces a slow voltage-gated potassium current. Science 1988;242:1042-1045.
12. Κs function. Nat Genet 1997;17:338-340.
13. Κs cardiac potassium current. Nature 1996;384:78-80.
14. Κs potassium channel. Nature 1996;384:80-83.
15. 15. Warmke JW, Ganetzky B: A family of potassium channel genes related to eag in Drosophila in mammals. Proc Natl Acad Sci USA 1994;91:3438-3442.
16. 16. Curran ME, Splawski I, Timothy KW, et al: A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell 1995;80:795-803.
17. Κr potassium channel. Cell 1995;81:299-307.
18. 18. Gellens ME, George AL Jr, Chen LQ, et al: Primary structure and functional expression of the human cardiac tetrodotoxin-insensitive voltage-dependent sodium channel. Proc Natl Acad Sci USA 1992;89:554-558.
19. 19. Wang Q, Shen J, Splawski I, et al: SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell 1995;80:805-811.
20. 20. Schott JJ, Charpentier F, Peltier S, et al: Mapping of a gene for long QT syndrome to chromosome 4q25-27. Am J Hum Genet 1995;57:1114-1122.
21. Κr potassium channels with HERG and is associated with cardiac arrhythmia. Cell 1999;97:175-187.
22. 22. Chouabe C, Neyroud N, Guicheney P, et al: Properties of KvLQT1 K+ channel mutations in Romano-Ward and Jervell and Lange-Nielsen inherited cardiac arrhythmias. EMBO J 1997;16:5472-5479.
23. + channel dysfunction in an inherited cardiac arrhythmia. Proc Natl Acad Sci USA 1996;93: 2208-2212.
24. 24. Romey G, Attali B, Chouabe C, et al: Molecular mechanism and functional significance of the mink control of the KvLQT1 channel activity. J Biol Chem 1997;272:16713-16716.
25. 25. Tai KK, Goldstein SN: The conduction pore of a cardiac potassium channel. Nature 1998;391:605-608.
26. + conduction and selectivity. Science 1998;280:69-77.
27. 27. MacKinnon R: Determination of the subunit stoichiometry of a voltage-activated potassium channel. Nature 1991;350:232-235.
28. 28. Zhou ZF, Gong QM, Epstein ML, et al: HERG channel dysfunction in human long QT syndrome - Intracellular transport and functional defects. J Biol Chem 1998;273:21061-21066.
29. + channel splice variant common in human heart lacks a C-terminal domain required for expression of rapidly-activating delayed rectifier current. J Biol Chem 1998; 273:27231-27235.
30. 30. Jiang M, Tseng-Crank J, Tseng GN: Suppression of slow delayed rectifier current by a truncated isoform of KvLQT1 cloned from normal human heart. J Biol Chem 1997;272:24109-24112.
31. 31. Demolombe S, Baro I, Pereon Y, et al: A dominant negative isoform of the long QT syndrome 1 gene product. J Biol Chem 1998;273:6837-6843.
32. + current. Circ Res 1997;81:870-878.
33. + channel in mouse and human hearts. Circ Res 1997;81:719-728.
34. 34. Li H, Chen Q, Moss AJ, et al: New mutations in the KVLQT1 potassium channel that cause long-QT syndrome. Circulation 1998;97:1264-1269.
35. 35. Mohammad-Panah R, Demolombe S, Neyroud N, et al: Mutations in a dominant-negative isoform correlate with phenotype in inherited cardiac arrhythmias. Am J Hum Genet 1999;64:1015-1023.
36. 36. Demolombe S, Drouin E, Baro I, et al: Expression of KvLQT1 in human ventricular epicardial, midmyocardial, and endocardial layers. (Abstract) Circulation 1998;98:I-610.
37. 37. Neyroud N, Tesson F, Denjoy I, et al: A novel mutation in the potassium channel gene KVLQT1 causes the Jervell and Lange-Nielsen cardioauditory syndrome. Nat Genet 1997;15:186-189.
38. 38. Schulzebahr E, Haverkamp W, Wedekind H, et al: Autosomal recessive long-QT syndrome (Jervell Lange-Nielsen syndrome) is genetically heterogeneous. Hum Genet 1997;100:573-576.
39. 39. Splawski I, Timothy KW, Vincent GM, et al: Molecular basis of the long QT syndrome associated with deafness. N Engl J Med 1997;336:1562-1567.
40. 40. Vetter DE, Mann JR, Wangemann P, et al: Inner ear defects induced by null mutation of the isk gene. Neuron 1996;17:1251-1264.
41. 41. Kupershmidt S, Yang T, Anderson ME, et al: Replacement by homologous recombination of the minK gene with IacZ reveals restriction of minK expression to the mouse cardiac conduction system. Circ Res 1999;84: 146-152.
42. 42. Priori SG, Schwartz PJ, Napolitano C, et al: A recessive variant of the Romano-Ward long-QT syndrome? Circulation 1998;97:2420-2425.
43. 43. Bennett PB, Yazawa K, Makita N, et al: Molecular mechanism for an inherited cardiac arrhythmia. Nature 1995;376:683-685.
44. + channel mutations in the congenital long QT syndrome. Proc Natl Acad Sci USA 1996;93:13200-13205.
45. 45. Dumaine R, Wang Q. Keating MT, et al: Multiple mechanisms of sodium channel-linked long QT syndrome. Circ Res 1996;78:916-924.
46. 46. Kambouris NG, Nuss HB, Johns DC, et al: Phenotypic characterization of a novel long-QT syndrome mutation (R1623Q) in the cardiac sodium channel. Circulation 1998;97:640-644.
47. 47. Wang DW, Wei J, Alings M, et al: A mutation (E1784K) in the carboxyl terminus of the cardiac Na+ channel causes autosomal dominant congenital long QT syndrome. (Abstract) Circulation 1998;98:I-56.
48. 48. Makita N, Shirai N, Nagashima M, et al: A de novo missense mutation of human cardiac Na+ channel exhibiting novel molecular mechanisms of long QT syndrome. FEBS Lett 1998;423:5-9.
49. 49. Nagatomo T, Fan Z, Ye B, et al: Temperature dependence of early and late currents in human wild-type and long QT ΔKPQ mutant sodium channels. Am J Physiol 1998;275:H2016-H2024.
50. 50. An RH, Wang XL, Kerem B, et al: Novel LQT-3 mutation affects Na+ channel activity through interactions between alpha-and beta 1-subunits. Circ Res 1998;83:141-146.
51. 51. Brugada J, Brugada R, Brugada P: Right bundlebranch block and ST-segment elevation in leads V-1 through V-3 - A marker for sudden death in patients without demonstrable structural heart disease. Circulation 1998;97:457-460.
52. 52. Nademanee K, Veerakul G, Nimmannit S, et al: Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men. Circulation 1997;96: 2595-2600.
53. 53. Chen QY, Kirsch GE, Zhang DM, et al: Genetic basis and molecular mechanism for idiopathic ventricular fibrillation. Nature 1998;392:293-296.
54. 54. Rook MB, Alshinawi C, Groenewegen WA, et al: Human SCN5A gene mutations alter Na-current kinetics and associate with VF in patients without structural heart disease. (Abstract) Circulation 1998;98:I-468.
55. 55. McNamara DM, Weiss R, Seibel J, et al: Molecular heterogeneity in the Brugada syndrome. (Abstract) Circulation 1998;98:I-456.
56. 56. Krishnan SC, Antzelevitch C: Sodium channel block produces opposite electrophysiological effects in canine ventricular epicardium and endocardium. Circ Res 1991;69:277-291.
57. 57. Brugada R, Tapscott T, Czernuszewicz GZ, et al: Identification of a genetic locus for familial atrial fibrillation. N Engl J Med 1997;336:905-911.
58. 58. Spirito P, Seidman CE, McKenna WJ, et al: The management of hypertrophic cardiomyopathy. N Engl J Med 1997;336:775-785.
59. 59. Olson TM, Michels VV, Thibodeau SN, et al: Actin mutations in dilated cardiomyopathy, a heritable form of heart failure. Science 1998;280:750-752.
60. 60. Corrado D, Basso C, Thiene G, et al: Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia - A multicenter study. J Am Coll Cardiol 1997;30:1512-1520.
61. 61. Coonar AS, Protonotarios N, Tsatsopoulou A, et al: Gene for arrhythmogenic right ventricular cardiomyopathy with diffuse nonepidermolytic palmoplantar keratoderma and woolly hair (Naxos disease) maps to 17q21. Circulation 1998;97:2049-2058.
62. 62. Saarinen K, Swan H, Kainulainen K, et al: Molecular genetics of the long QT syndrome: Two novel mutations of the KVLQT1 gene and phenotypic expression of the mutant gene in a large kindred. Hum Mutat 1998;11:158-165.
63. 63. Vincent GM, Timothy KW, Leppert M, et al: The spectrum of symptoms and QT intervals in carriers of the gene for the long-QT syndrome. N Engl J Med 1992;327:846-852.
64. 64. Lehmann MH, Timothy KW, Frankovich D, et al: Age-gender influence on the rate-corrected QT interval and the QT-heart rate relation in families with genotypically characterized long QT syndrome. J Am Coll Cardiol 1997;29:93-99.
65. 65. Priori SG, Napolitano C, Schwartz PJ: Low penetrance in the long-QT syndrome: Clinical impact. Circulation 1999;99:529-533.
66. 66. Priori SG, Napolitano C, Brown AM, et al: The loss of function induced by HERG and KVLQT1 mutations does not correlate with the clinical severity of the long QT syndrome. (Abstract) Circulation 1998;98:I-457.
67. 67. Wang Z, Tristani-Firouzi M, Xu Q, et al: Functional effects of mutations in KvLQT1 that cause long QT syndrome. J Cardiovasc Electrophysiol 1999;10:817-826.
68. 68. Zareba W, Moss AJ, Schwartz PJ, et al: Influence of the genotype on the clinical course of the long-QT syndrome. N Engl J Med 1998;339:960-965.
69. 69. Locati EH, Zareba W, Moss AJ, et al: Age-and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: Findings from the International LQTS Registry. Circulation 1998;97:2237-2244.
70. 70. Donger C, Denjoy I, Berthet M, et al: KVLQT1 C-terminal missense mutation causes a forme fruste long-QT syndrome. Circulation 1997;96:2778-2781.
71. 71. Napolitano C, Priori SG, Schwartz PJ, et al: Identification of a long QT syndrome molecular defect in drug-induced torsades de pointes. (Abstract) Circulation 1997;96:I-211.
72. 72. Schulze-Bahr E, Haverkamp W, Hordt M, et al: Do mutations in cardiac ion channel genes predispose to drug-induced (acquired) long QT syndrome? (Abstract) Circulation 1997;96:I-211.
73. 73. Roden DM: Taking the idio out of idiosyncratic -Predicting torsades de pointes. PACE 1998;21:1029-1034.
74. 74. Hajj-Ali R, Zareba W, Moss AJ, et al: The differentiating features of the Romano-Ward and the Jervell and Lange-Nielsen syndromes. (Abstract) Circulation 1997;96:I-153.
75. 75. Moss AJ, Zareba W, Benhorin J, et al: ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation 1995;92:2929-2934.
76. 76. Dausse E, Berthet M, Denjoy I, et al: A mutation in HERG associated with notched T waves in long QT syndrome. J Mol Cell Cardiol 1996;28:1609-1615.
77. 77. Guicheney P, Berthet M, Donger C, et al: Morphological abnormalities of the T wave on Holter recordings have a diagnostic but not a prognostic value. (Abstract) Circulation 1998;I-672.
78. 78. Priori SG, Mortara DW, Napolitano C, et al: Evaluation of the spatial aspects of T-wave complexity in the long-QT syndrome. Circulation 1997;96:3006-3012.
79. 79. Zareba W, Moss AJ, Konecki J: TU wave area-derived measures of repolarization dispersion in the long QT syndrome. J Electrocardiol 1998;30(Suppl):191-195.
80. 80. Schwartz PJ, Moss AJ, Priori SG, et al: Gene-specific influence on the triggers for cardiac arrest in the long QT syndrome. (Abstract) Circulation 1997;96:I-212.
81. + channel blockade and to increases in heart rate: Implications for gene-specific therapy. Circulation 1995;92: 3381-3386.
82. 82. Moss AJ, Schwartz PJ, Crampton RS, et al: The long QT syndrome. Prospective longitudinal study of 328 families. Circulation 1991;84:1136-1144.
83. 83. Priori SG, Napolitano C, Schwartz PJ, et al: The risk of sudden death as first cardiac event in asymptomatic patients with the long QT syndrome. (Abstract) Circulation 1998;98:I-777.
84. 84. Wilde AA, Jongbloed RJ, Doevendans PA, et al: Auditory stimuli as a trigger for arrhythmic events differentiate HERG-related (LQTS2) patients from KVLQT1-related patients (LQTS1). J Am Coll Cardiol 1999;33:327-332.
85. 85. Rautaharju PM, Zhou SH, Wong S, et al: Sex differences in the evolution of the electrocardiographic QT interval with age. Can J Cardiol 1992;8:690-695.
86. 86. Makkar RR, Fromm BS, Steinman RT, et al: Female gender as a risk factor for torsades de pointes associated with cardiovascular drugs. JAMA 1993;270: 2590-2597.
87. 87. Drici MD, Burklow TR, Haridasse V, et al: Sex hormones prolong the QT interval and down regulate potassium channel expression in the rabbit heart. Circulation 1996;94:1471-1474.
88. 88. Tsunoda S, Sierralta J, Sun Y, et al: A multivalent PDZ-domain protein assembles signalling complexes in a G-protein-coupled cascade. Nature 1997;388:243-249.
89. 89. Wible BA, Yang Q, Kuryshev YA, et al: Cloning and expression of a novel K+ channel regulatory protein, KChAP. J Biol Chem 1998;273:11745-11751.
90. 90. Accili EA, Kiehn J, Yang Q, et al: Separable Kv-beta subunit domains alter expression and gating of potassium channels. J Biol Chem 1997;272:25824-25831.
91. + channel beta subunit expressed in human heart. Proc Natl Acad Sci USA 1995;92:6309-6313.
92. 92. Murray KT, Hu N, England SK, et al: Coexpression of a beta subunit enhances the effect of a phorbol ester on the Kv1.5 channel. (Abstract) Circulation 1996;94:I-473.
93. 93. Turki J, Pak J, Green SA, et al: Genetic polymorphisms of the beta 2-adrenergic receptor in nocturnal and nonnocturnal asthma. Evidence that Gly 16 correlates with the nocturnal phenotype. J Clin Invest 1995; 95:1635-1641.
94. 94. Lee MP, Hu RJ, Johnson LA, et al: Human KvLQT1 gene shows tissue-specific imprinting and encompasses Beckwith-Wiedemann syndrome chromosomal rearrangements. Nat Genet 1997;15:181-185.
95. 95. Shi W, Wymore RS, Wang HS, et al: Identification of two nervous system-specific members of the erg potassium channel gene family. J Neurosci 1997;17: 9423-9432.
96. 96. Marks ML, Trippel DL, Keating MT: Long QT syndrome associated with syndactyly identified in females. Am J Cardiol 1995;76:744-745.
97. 97. Rosero SZ, Zareba W, Robinson JL, et al: Asthma in the long QT syndrome: A modifier of cardiac morbidity. (Abstract) Circulation 1997;96:I-212.
98. 98. Wang DW, Yazawa K, Makita N, et al: Pharmacological targeting of long QT mutant sodium channels. J Clin Invest 1997;99:1714-1720.
99. + channels. Circ Res 1996;79:103-108.
100. 100. Rosero SZ, Zareba W, Hall WJ, et al: Gene-specific therapy for long QT syndrome: QT shortening with lidocaine and tocainide in patients with mutation of the sodium-channel gene. Ann Noninvas Electrocardiogr 1997;2:274-278.
101. 101. Roden DM, Iansmith DH, Woosley RL: Frequency-dependent interactions of mexiletine and quinidine on depolarization and repolarization in canine Purkinje fibers. J Pharmacol Exp Ther 1987;243:1218-1224.
102. 102. Chezalviel-Guilbert F, Davy JM, Poirier JM, et al: Mexiletine antagonizes effects of sotalol on QT interval duration and its proarrhythmic effects in a canine model of torsade de pointes. J Am Coll Cardiol 1995; 26:787-792.
103. 103. Sicouri S, Antzelevitch D, Heilmann C, et al: Effects of sodium channel block with mexiletine to reverse action potential prolongation in in vitro models of the long term QT syndrome. J Cardiovasc Electrophysiol 1997;8:1280-1290.
104. 104. Shimizu W, Antzelevitch C: Sodium channel block with mexiletine is effective in reducing dispersion of repolarization and preventing torsade des pointes in LQT2 and LQT3 models of the long-QT syndrome. Circulation 1997;96:2038-2047.
105. + currents. Mol Pharmacol 1998;54:220-230.
106. 106. Tseng GN, Zhu B, Ling S, et al: Quinidine enhances and suppresses Kv1.2 from outside and inside the cell, respectively. J Pharmacol Exp Ther 1996;279:844-855.
107. 107. Shimizu W, Kurita T, Matsuo K, et al: Improvement of repolarization abnormalities by a K+ channel opener in the LQT1 form of congenital long-QT syndrome. Circulation 1998;97:1581-1588.
108. 108. Sato T, Hata Y, Yamamoto M, et al: Early afterdepolarization abolished by potassium channel opener in a patient with idiopathic long QT syndrome. J Cardiovasc Electrophysiol 1995;6:279-282.
109. 109. Chinushi M, Aizawa Y, Furushima H, et al: Nicorandil suppresses a hump on the monophasic action potential and torsade de pointes in a patient with idiopathic long QT syndrome. Jpn Heart J 1995;36:477-481.
110. + channels. Neuron 1996;16:1011-1017.
111. 111. Babenko AP, Aguilar-Bryan L, Bryan J: A view of sur/KIR6.X, KATP channels. Annu Rev Physiol 1998; 60:667-687.
112. + currents. Pflugers Arch 1992;420:180-186.
113. Κr: Implications for torsades de pointes and reverse use-dependence. Circulation 1996; 93:407-411.
114. + current in cardiac cells. Circ Res 1997;80:782-789.
115. 115. Compton SJ, Lux RL, Ramsay MR, et al: Genetically-defined therapy of inherited long-QT syndrome: Correction of abnormal repolarization by potassium. Circulation 1996;94:1018-1022.
116. 116. Choy AM, Lang CC, Chomsky DM, et al: Normalization of acquired QT prolongation in humans by intravenous potassium. Circulation 1997;96:2149-2154.
117. 117. Dorostkar PC, Eldar M, Belhassen B, et al: Long-term follow-up of patients with the idiopathic long-QT syndrome treated with combined beta-blockers and chronic pacing. (Abstract) Circulation 1997;96:I-212.
118. 118. Schwartz PJ, Periti M, Malliani A: The long QT syndrome. Am Heart J 1975;89:378-390.
119. 119. Anderson ME, Braun AP, Wu Y, et al: KN-93, an inhibitor of multifunctional Ca2+/calmodulin dependent protein kinase, decreases early afterdepolarizations in rabbit heart. J Pharmacol Exp Ther 1998;287: 996-1006.
120. 120. Splawski I, Shen J, Timothy KW, et al: Genomic structure of three long QT syndrome genes: KVLQT1, HERG, and KCNE1. Genomics 1998;51:86-97.
121. 121. Itoh T, Tanaka T, Nagai R, et al: Genomic organization and mutational analysis of HERG, a gene responsible for familial long QT syndrome. Hum Genet 1998;102:435-439.
122. 122. Wang Q, Li Z, Shen J, et al: Genomic organization of the human SCN5A gene encoding the cardiac sodium channel. Genomics 1996;34:9-16.
123. 123. Dangman KH, Hoffman BF: In vivo and in vitro antiarrhythmic and arrhythmogenic effects of N-acetyl procainamide. J Pharmacol Exp Ther 1981;217:851-862.
124. 124. Brachmann J, Scherlag BJ, Rosenshtraukh LV, et al: Bradycardia-dependent triggered activity: Relevance to drug-induced multiform ventricular tachycardia. Circulation 1983;68:846-856.
125. 125. Roden DM, Hoffman BF: Action potential prolongation and induction of abnormal automaticity by low quinidine concentrations in canine Purkinje fibers. Relationship to potassium and cycle length. Circ Res 1985;56:857-867.
126. 126. 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 1996;79:474-492.
127. 127. El-Sherif N, Chinushi M, Caref EB, et al: 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 1997;96:4392-4399.
128. 128. Asano Y, Davidenko JM, Baxter WT, et al: Optical mapping of drug-induced polymorphic arrhythmias and torsades de pointes in the isolated rabbit heart. J Am Coll Cardiol 1997;29:831-842.
129. 129. Hii JT, Wyse DG, Gillis AM, et al: Precordial QT interval dispersion as a marker of torsade de pointes. Disparate effects of class Ia antiarrhythmic drugs and amiodarone. Circulation 1992;86:1376-1382.
130. 130. Day CP, McComb JM, Campbell RWF: QT dispersion: An indication of arrhythmia risk in patients with long QT intervals. Br Heart J 1990;63:342-344.
131. 131. Verduyn SC, Vos MA, Vanderzande J, et al: Further observations to elucidate the role of interventricular dispersion of repolarization and early afterdepolarizations in the genesis of acquired torsade de pointes arrhythmias - A comparison between almokalant and d-sotalol using the dog as its own control. J Am Coll Cardiol 1997;30:1575-1584.
132. 132. Lazzara R: Amiodarone and torsades de pointes. Ann Intern Med 1989;111:549-551.
133. 133. Sicouri S, Moro S, Litovsky S, et al: Chronic amiodarone reduces transmural dispersion of repolarization in the canine heart. J Cardiovasc Electrophysiol 1997; 8:1269-1279.
134. 134. Cowan JC, Yusoff K, Moore M, et al: Importance of lead selection in QT interval measurement. Am J Cardiol 1988;61:83-87.
135. 135. Berger RD, Kasper EK, Baughman KL, et al: Beatto-beat QT interval variability - Novel evidence for repolarization lability in ischemic and nonischemic dilated cardiomyopathy. Circulation 1997;96:1557-1565.
136. 2+ current. Circ Res 1989;64:977-990.
137. 137. Attwell D, Cohen I, Eisner D, et al: The steady state TTX-sensitive ("window") sodium current in cardiac Purkinje fibers. Pflugers Arch 1979;379:137-142.
138. 138. Hirano Y, Moscucci A, January CT: Direct measurement of L-type Ca2+ window current in heart cells. Circ Res 1992;70:445-455.
139. 139. 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 1995;26:1299-1309.
140. +-induced early afterdepolarizations in Purkinje fibers. J Cardiovasc Electrophysiol 1994;5:933-944.
141. 141. Xu X, Lee KS: A selective blocker for rested T-type Ca++ channels in guinea pig atrial cells. J Pharmacol Exp Ther 1994;268:1135-1142.
142. 142. Ertel SI, Ertel EA, Clozel JP: T-type Ca2+ channels and pharmacological blockade: Potential pathophysiological relevance. Cardiovasc Drugs Ther 1997;11: 723-739.
143. 143. Bouchard RA, Clark RB, Giles WR: Effects of action potential duration on excitation-contraction coupling in rat ventricular myocytes. Action potential voltage-clamp measurements. Circ Res 1995;76:790-801.
144. 144. Wu Y, Roden DM, Anderson ME: CaM kinase inhibition prevents development of the arrhythmogenic transient inward current. Circ Res 1999;84:906-912.
145. 145. Wu Y, Macmillan LB, McNeill RB, et al: CaM kinase augments cardiac L-type Ca2+ current: A cellular mechanism for long Q-T arrhythmias. Am J Physiol 1999;276:H2168-H2178.
146. 146. Wu Y, Roden DM, Anderson ME: Calmodulin kinase inhibition prevents development of the arrhythmogenic transient inward current. Circ Res 1999:84:906-912.
147. 147. Mazur A, Roden DM, Anderson ME: Torsade de pointes is prevented by systemic administration of a calmodulin antagonist. (Abstract) Circulation 1998; 98:I-814.
148. + current by dofetilide. Circ Res 1993;72:75-83.
149. 149. Sanguinetti MC, Jurkiewicz NK, Scott A, et al: Isoproterenol antagonizes prolongation of refractory period by the class III antiarrhythmic agent E-4031 in guinea pig myocytes: Mechanism of action. Circ Res 1991;68:77-84.
150. 150. Hondeghem LM, Snyders DJ: Class III antiarrhythmic agents have a lot of potential, but a long way to go: Reduced effectiveness and dangers of reverse use-dependence. Circulation 1990;81:686-690.
151. 151. Antzelevitch C, Sicouri S, Lukas A, et al: Regional differences in the electrophysiology of ventricular cells: Physiological and clinical implications. In Zipes DP, Jalife J, eds: Cardiac Electrophysiology: From Cell to Bedside. WB Saunders, Philadelphia, 1995, pp. 228-245.
152. Κs contributes to the longer action potential of the M cell. Circ Res 1995;76:351-365.
153. + channel mRNA in isolated ferret cardiac myocytes. Circ Res 1996;78:1083-1089.
154. + channel currents in rat ventricular myocytes. J Gen Physiol 1996;108:405-419.
155. + channel cDNAs from human ventricle. FASEB J 1991;5:331-337.
156. + channel protein in explanted cardiac tissue. J Clin Invest 1995;96:282-292.
157. 157. Cohen SA: Immunocytochemical localization of rH1 sodium channel in adult rat heart atria and ventricle: Presence in terminal intercalated disks. Circulation 1996;94:3083-3086.
158. 158. Mays DJ, Boyden PA, Tamkun MM: Redistribution of the Kv1.5 potassium channel protein on the surface of myocytes from the epicardial border zone of the infarcted canine heart. Cardiovasc Pathobiol 1997;2:79-87.
159. + channel in heart rate control of repolarization in a murine engineered model of Jervell and Lange-Nielsen Syndrome. Circ Res 1998;83:95-102.
160. 160. Folco E, Mathur R, Mori Y, et al: A cellular model for long QT syndrome - Trapping of heteromultimeric complexes consisting of truncated Kv1.1 potassium channel polypeptides and native kv1.4 and kv1.5 channels in the endoplasmic reticulum. J Biol Chem 1997;272:26505-26510.
161. 161. Barry DM, Xu HD, Schuessler RB, et al: Functional knockout of the transient outward current, long-QT syndrome, and cardiac remodeling in mice expressing a dominant-negative Kv4 subunit. Circ Res 1998;83: 560-567.
162. 162. Wickenden AD, Huang Q, Factor SM, et al: Expression of a dominant negative Kv4.2 channel in the hearts of transgenic mice. (Abstract) Circulation 1998; 96:I-422.
163. + current by overexpression of the long-QT syndrome HERG G628S mutation in transgenic mice. Circ Res 1998;83:668-678.
164. 164. Hart G: Cellular electrophysiology in cardiac hypertrophy and failure. Cardiovasc Res 1994;28:933-946.
165. 165. Nabauer M, Kaab S: Potassium channel down-regulation in heart failure. Cardiovasc Res 1998;37:324-334.
166. + currents in isolated human ventricular myocytes from patients with terminal heart failure. Circ Res 1993;73:379-385.
167. 167. Kääb S, Nuss HB, Chiamvimonvat N, et al: Ionic mechanism of action potential prolongation in ventricular myocytes from dogs with pacing-induced heart failure. Circ Res 1996;78:262-273.
168. 168. Kaab S, Dixon J, Duc J, et al: Molecular basis of transient outward potassium current downregulation in human heart failure: A decrease in Kv4.3 mRNA correlates with a reduction in current density. Circulation 1998;98:1383-1393.
169. 169. Tomaselli GF, Beuckelmann DJ, Calkins HG, et al: Sudden cardiac death in heart failure. The role of abnormal repolarization. Circulation 1994;90:2534-2539.
170. 170. Schroder F, Handrock R, Beuckelmann DJ, et al: Increased availability and open probability of single L-type calcium channels from failing compared with nonfailing human ventricle. Circulation 1998;98:969-976.
171. 2+ current is expressed in hypertrophied adult feline left ventricular myocytes. Circ Res 1993;73:777-782.
172. 172. Maltsev VA, Sabbah HN, Higgins RS, et al: Novel, ultraslow inactivating sodium current in human ventricular cardiomyocytes. Circulation 1998;98:2545-2552.
173. 2+ exchanger in end-stage human heart failure. Circ Res 1994;75:443-453.
174. 174. Lue WM, Boyden PA: Abnormal electrical properties of myocytes from chronically infarcted canine heart. Alterations in Vmax and the transient outward current. Circulation 1992;85:1175-1188.
175. 175. Ursell PC, Gardner PI, Albala A, et al: Structural and electrophysiological changes in the epicardial border zone of canine myocardial infarcts during infarct healing. Circ Res 1985;56:436-451.
176. 176. Peters NS, Coromilas J, Severs NJ, et al: Disturbed Connexin43 gap junction distribution correlates with the location of reentrant circuits in the epicardial border zone of healing canine infarcts that cause ventricular tachycardia. Circulation 1997;95:988-996.
177. 177. Peters NS: New insights into myocardial arrhythmogenesis: Distribution of gap-junctional coupling in normal, ischaemic and hypertrophied human hearts. Clin Sci 1996;90:447-452.
178. 178. Yao JA, Peters NS, Boyden PA, et al: Reduced electrical coupling between ventricular cell pairs from the epicardial border zone of canine myocardial infarcts. (Abstract) Circulation 1998;98:I-54.
179. 179. Pinto JM, Boyden PA: Electrical remodeling in ischemia and infarction. Cardiovasc Res 1999;42: 284-297.
180. + current density in arrhythmogenic subendocardial Purkinje myocytes from the infarcted heart. J Cardiovasc Electrophysiol 1998; 9:299-311.
181. 181. Zeiler RH, Gough WB, El-Sherif N: Electrophysiologic effects of propafenone on canine ischemic cardiac cells. Am J Cardiol 1984;54:424-429.
182. 182. Aggarwal R, Pu J, Boyden PA: Ca(2+)-dependent outward currents in myocytes from epicardial border zone of 5-day infarcted canine heart. Am J Physiol 1997;273:H1386-H1394.
183. 183. Kalman D, O'Lague PH, Erxleben C, et al: Calcium-dependent inactivation of the dihydropyridine-sensitive calcium channels in GH3 cells. J Gen Physiol 1988;92:531-548.
184. 184. Zygmunt AC, Gibbons WR: Calcium-activated chloride current in rabbit ventricular myocytes. Circ Res 1991;68:424-437.
185. 185. Dolmetsch RE, Xu KL, Lewis RS: Calcium oscillations increase the efficiency and specificity of gene expression. Nature 1998;392:933-936.