Congenital Long QT Syndrome

Cardiac Electrophysiology Review

Volumn 3, Issue 3, 1999, Pages 207-209

  Vincent, G Michael ^a,b   Timothy, Katherine ^a   Zhang, Li ^a  

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

^b Department of Medicine ^*  (United States)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 0000657921     PISSN: 13852264     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1009987508571     Document Type: Article

References (14)

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2. Sanguinetti MC, Curran ME, Zou A, Shen J, Spector PS, Atkinson DL, Keating MT. Co-assembly of KvLQT1 and minK (IsK) proteins to form cardiac Iks potassium channel. Nature 1996;384:80-83.
3. Abbott GW, Sesti F, Splawski I, Buck ME, Lehmann MH, Timothy KW, Keating MT, Goldstein SA. MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmias. Cell 1999;97:175-187.
4. Splawski I, Shen J, Timothy KW, Vincent GM, Lehmann MH, Keating MT. Genomic structure of three long QT syndrome genes: KVLQT1, HERG, and KCNE1. Genomics 1998;51:86-97.
5. Schulze-Bahr E, Wang Q, Wedekind H, Haverkamp W, Chen Q, Sun Y. KCNE1 mutations cause Jervell and Lange-Nielsen syndrome. Nat Genet 1997;17:267-268.
6. Splawski I, Timothy KW, Vincent GM, Atkinson DL, Keating MT. Molecular basis of the long-QT syndrome associated with deafness. Proc Assoc Am Physicians 1997;109:504-511.
7. Neyroud N, Tesson F, Denjoy I, Leibovici M, Donger C, Barhanin J, Faure S, Gary F, Coumel P, Petit C, Schwartz K, Guicheney P. A novel mutation in the potassium channel gene KVLQT1 causes the Jervell and Lange-Nielsen cardioauditory syndrome. Nat Genet 1997;15:186-189.
8. 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 1997;96:2038-2047.
9. Shimizu W, Antzelevitch C. Cellular basis for the ECG features of the LQT1 form of the long-QT syndrome: Effects of beta-adrenergic agonists and antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes. Circulation 1998;98:2314-2322.
10. Vincent GM, Timothy KW, Leppert M, Keating MT. The spectrum of symptoms and QT intervals in the carriers of the gene for the long-QT syndrome. N Engl J Med 1992; 327:846-852.
11. Guili LC, Zhang L, Timothy KW, Splawski I, Shen J, Fox J, Keating M, Vincent GM. Long QT genotype can be identified by ECG phenotype. J Am Coll Cardiol 1998;31:192A.
12. Zhang L, Timothy KW, Splawski I, Shen J, Fox J, Keating M, Vincent GM. Genotypes are highly predictable in long QT syndrome families with typical ECG ST-T wave patterns. Circulation 1998;98:17A.
13. Compton SJ, Lux RL, Ramsey MR, Strelich KR, Sanguinetti MC, Green LS, Keating MT, Mason JW. Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium. Circulation 1996; 94:1018-1022.
14. Tan HL, Alings M, VanOlden RW, Wilde AAM. Long-term (subacute) potassium treatment in congenital HERG-related long QT syndrome (LQTS2). J Cardiovasc Electrophysiol 1999;10:229-233.