Genetically defined therapy of inherited long-QT syndrome: Correction of abnormal repolarization by potassium

Circulation

Volumn 94, Issue 5, 1996, Pages 1018-1022

  Compton, Steve J ^a   Lux, Robert L ^a   Ramsey, Matthew R ^a   Strelich, Katie R ^a   Sanguinetti, Michael C ^a   Green, Larry S ^a   Keating, Mark T ^a,b   Mason, Jay W ^a,c  

^a UNIVERSITY OF UTAH   (United States)

^b UNIVERSITY OF UTAH SCHOOL OF MEDICINE   (United States)

^c UNIVERSITY OF UTAH HEALTH SCIENCES CENTER   (United States)

Author keywords

genes; long QT syndrome; potassium

Indexed keywords

POTASSIUM CHANNEL; POTASSIUM CHLORIDE;

ADULT; ARTICLE; CHROMOSOME 7; CLINICAL ARTICLE; FEMALE; GENE MUTATION; HEART PACING; HEART REPOLARIZATION; HUMAN; LONG QT SYNDROME; MALE; PATHOGENESIS; POTASSIUM BLOOD LEVEL; POTASSIUM CURRENT; PRIORITY JOURNAL; T WAVE;

EID: 0029831629     PISSN: 00097322     EISSN: None     Source Type: Journal    
DOI: 10.1161/01.CIR.94.5.1018     Document Type: Article

References (40)

1. Romano C, Gemme G, Pongiglione R. Aritmie cardiache rare dell'eta pediatrica. Clin Pediatr. 1963;45:656-683.
2. Jervell A, Lange-Nielsen F. Congenital deaf mutism, functional heart disease with prolongation of the Q-T interval and sudden death. Am Heart J. 1957;54:59-68.
3. Ward OC. A new familial cardiac syndrome in children. J Irish Med Assoc. 1964:54:103-106.
4. Tan HL, Hou CJY, Lauer MR, Sung RJ. Electrophysiologic mechanisms of the long QT interval syndromes and torsade de pointes. Ann Intern Med. 1995:122:701-714.
5. Linker NJ, Colonna P, Kekwick CA, Till J, Camm AJ, Ward DE. Assessment of QT dispersion in symptomatic patients with congenital long QT syndromes. Am J Cardiol. 1992;69:634-638.
6. 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.
7. Priori SG, Napolitano C, Diehl L, Schwartz PJ. Dispersion of the QT interval: a marker of therapeutic efficacy in the idiopathic long QT syndrome. Circulation. 1994;89:1681-1689.
8. Benhorin J, Merri M, Alberti M, Locati E, Moss A, Hall W, Cui L. Long QT syndrome: new electrocardiographic characteristics. Circulation. 1990;82:521-527.
9. Malfatto G, Beria G, Sala S, Bonazzi O, Schwartz PJ. Quantitative analysis of T wave abnormalities and their prognostic implications in the idiopathic long QT syndrome. J Am Coll Cardiol. 1994;23: 296-301.
10. Schwartz PJ, Malliani A. Electrical alternation of the T-wave: clinical and experimental evidence of its relationship with the sympathetic nervous system and with the long QT syndrome. Am Heart J. 1975;89:45-50.
11. Vincent GM, Jaiswal D, Timothy KW. Effects of exercise on heart rate, QT, QTc and QT/QS2 in the Romano-Ward inherited long QT syndrome. Am J Cardiol. 1991;68:498-503.
12. Shimizu W, Ohe T, Kurita T, Shimomura K. Differential response of QTU interval to exercise, isoproterenol, and atrial pacing in patients with congenital long QT syndrome. Pacing Clin Electrophysiol. 1991;14:1966-1970.
13. Merri M, Moss AJ, Benhorin J, Locati EH, Alberti M, Badilini F. Relation between ventricular repolarization duration and cardiac cycle length during 24-hour Holter recordings: findings in normal patients and patients with long QT syndrome. Circulation. 1992; 85:1816-1821.
14. De Ambroggi L, Bertoni T, Locati E, Stramba-Badiale M, Schwartz PJ. Mapping of body surface potentials in patients with the idiopathic long QT syndrome. Circulation. 1986;74:1334-1345.
15. Moss AJ, Schwartz PJ, Crampton RS, Locati E, Carleen E. The long QT syndrome: a prospective international study. Circulation. 1985; 71:17-21.
16. Schwartz PH, Locati E. The idiopathic long QT syndrome: pathogenetic mechanisms and therapy. Eur Heart J. 1985;6(suppl D): 103-114.
17. Moss AJ, Lieu JE, Gottlieb S, Locati EH, Schwartz PJ, Robinson JL. Efficacy of permanent pacing in the management of high-risk patients with long QT syndrome. Circulation. 1991;84:1524-1529.
18. Eldar M, Griffin JC, VanHare GF, Witherell C, Bhandari A, Benditt D, Scheinman M. Combined use of beta-adrenergic blocking agents and long-term cardiac pacing for patients with the long QT syndrome. J Am Coll Cardiol. 1992;20:830-837.
19. Schwanz PJ, Locati EH, Moss AJ, Crampton RS, Trazzi R, Ruberti U. Left cardiac sympathetic denervation in the therapy of congenital long QT syndrome: a worldwide report. Circulation. 1991;84: 503-511.
20. Curran, ME, Splawski I, Timothy KW, Vincent GM, Green ED, Keating MT. A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell. 1995;80:795-803.
21. Warmke JE, Ganetzky B. A family of potassium channel genes related to eag in Drosophila and mammals. Proc Natl Acad Sci U S A. 1994;91:3438-3442.
22. + currents. Pflugers Arch. 1992; 420:180-186.
23. Kr potassium channel. Cell. 1995;81:299-307.
24. Green LS, Lux RL, Haws CW, Williams RR, Hunt SC, Burgess MJ. Effects of age, sex, and body habitus on QRS and ST-T potential maps of 1100 normal subjects. Circulation. 1985;71:244-253.
25. Statters DJ, Malik M, Ward DE, Camm AJ. QT dispersion: problems of methodology and clinical significance. J Cardiovasc Electrophysiol. 1994;5:672-685.
26. Bazett HC. An analysis of the time-relations of electrocardiograms. Heart. 1920;7:353.
27. + channel dysfunction in an inherited cardiac arrhythmia. Proc Natl Acad Sci U S A. 1996;93:2208-2212.
28. + channel blockade and to increases in heart rate: implications for gene-specific therapy. Circulation. 1995;92:3381-3386.
29. Waber LJ, Valle D, Neill C, DiMauro S, Shug A. Carnitine deficiency presenting as familial cardiomyopathy: a treatable defect in carnitine transport. J Pediatr. 1982;101:700-705.
30. Cecchetti G, Binda A, Piperno A, Nador F, Fargion S, Fiorelli G. Cardiac alterations in 36 consecutive patients with idiopathic haemochromatosis: polygraphic and echocardiographic evaluation. Eur Heart J. 1991;12:224-230.
31. Leitersdorf E, Eisenberg S, Eliav O, Friedlander Y, Berkman N, Dann E, Landsberger D, Sehayek E, Meiner V, Wurm M, Bard J-M, Fruchart J-C, Stein Y. Genetic determinants of responsiveness to the HMG-CoA reductase inhibitor fluvastatin in patients with molecularly defined heterozygous familial hypercholesterolemia. Circulation. 1993;87(suppl III): III-35-III-44.
32. Schwartz PJ, Moss AJ, Vincent GM, Crampton RS. Diagnostic criteria for the long QT syndrome: an update. Circulation. 1993;88: 782-784.
33. Zhang L, Timothy K, Fox J, Vincent GM. Beta-Mockers modify T-wave morphology in long QT syndrome. J Am Coll Cardiol. 1993; 21:406A. Abstract.
34. Nador F, Beria G, De Ferrari GM, Stramba-Badiale M, Locati E, Lotto A, Schwartz P. Unsuspected echocardiographic abnormality in the long QT syndrome: diagnostic, prognostic, and pathogenetic implications. Circulation. 1991;84:1530-1542.
35. Fu Z, Timothy K, Fox J, Vincent GM. Beta-blockers markedly affect QT dispersion during exercise in long QT syndrome patients. J Am Coll Cardiol. 1993;21:93A. Abstract.
36. Keating MT, Atkinson D, Dunn C, Timothy K, Vincent GM, Leppert M. Linkage of a cardiac arrhythmia, the long QT syndrome, and the Harvey ras-1 gene. Science. 1991;252:704-706.
37. Jiang C, Atkinson D, Towbin JA, Splawski I, Lehmann M, Li H, Timothy K, Taggart R, Schwartz P, Vincent G, Moss A, Keating M. Two long QT syndrome loci map to chromosomes 3 and 7 with evidence for further heterogeneity. Nat Genet. 1994;8:141-147.
38. Schott JJ, Charpentier F, Peltier S, Foley P, Drouin E, Bouhour JB, Donnelly P. Mapping of a gene for long QT syndrome to chromosome 4q25-27. Am J Hum Genet. 1995;57:1114-1122.
39. Wang Q, Shen J, Splawski I, Atkinson D, Lie Z, Robinson J, Moss A, Towbin J, Keating M. SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell. 1995;80: 805-811.
40. Kr: implications for torsade de pointes and reverse use dependence. Circulation. 1996;93:407-411.