Utility of treadmill testing in identification and genotype prediction in long-QT syndrome

Circulation: Arrhythmia and Electrophysiology

Volumn 3, Issue 2, 2010, Pages 120-125

  Wong, Jorge A ^a   Gula, Lorne J ^a   Klein, George J ^a   Yee, Raymond ^a   Skanes, Allan C ^a   Krahn, Andrew D ^a  

^a UNIVERSITY OF WESTERN ONTARIO   (Canada)

Author keywords

Diagnosis; Exercise; Long QT syndrome; QT interval

Indexed keywords

ATENOLOL; BETA ADRENERGIC RECEPTOR BLOCKING AGENT; BISOPROLOL; ERG1 POTASSIUM CHANNEL; KCNE1 PROTEIN, HUMAN; KCNE2 PROTEIN, HUMAN; KCNQ1 PROTEIN, HUMAN; MUSCLE PROTEIN; POTASSIUM CHANNEL HERG; POTASSIUM CHANNEL KCNQ1; SCN5A PROTEIN, HUMAN; SODIUM CHANNEL; VOLTAGE GATED POTASSIUM CHANNEL;

ADULT; ARTICLE; CONTROLLED STUDY; DRUG DOSE TITRATION; ELECTROCARDIOGRAM; FEMALE; GENE MUTATION; GENETIC ANALYSIS; GENOTYPE; HUMAN; HYSTERESIS; LONG QT SYNDROME; MAJOR CLINICAL STUDY; MALE; PHENOTYPE; PREDICTION; PRIORITY JOURNAL; PROVOCATION TEST; QT INTERVAL; QT PROLONGATION; RISK ASSESSMENT; RR INTERVAL; TREADMILL EXERCISE; ADOLESCENT; BODY POSTURE; CLINICAL TRIAL; ELECTROCARDIOGRAPHY; EXERCISE; EXERCISE TEST; GENETICS; MIDDLE AGED; PREDICTION AND FORECASTING; STANDARD; VALIDATION STUDY;

ADOLESCENT; ADRENERGIC BETA-ANTAGONISTS; ADULT; ATENOLOL; ELECTROCARDIOGRAPHY; ETHER-A-GO-GO POTASSIUM CHANNELS; EXERCISE; EXERCISE TEST; FEMALE; GENOTYPE; HUMANS; KCNQ1 POTASSIUM CHANNEL; LONG QT SYNDROME; MALE; MIDDLE AGED; MUSCLE PROTEINS; PHENOTYPE; POSTURE; POTASSIUM CHANNELS, VOLTAGE-GATED; PREDICTIVE VALUE OF TESTS; REFERENCE STANDARDS; SODIUM CHANNELS; YOUNG ADULT;

EID: 77951918931     PISSN: 19413149     EISSN: 19413084     Source Type: Journal    
DOI: 10.1161/CIRCEP.109.907865     Document Type: Article

References (22)

1. Ackerman MJ, Clapham DE. Mechanisms of disease: ion channels: basic science and clinical disease. N Engl J Med. 1997; 336: 1575-1586.
2. Schwartz PJ, Moss AJ, Vincent GM, Crampton RS. Diagnostic criteria for the long-QT syndrome: an update. Circulation. 1993; 88: 782-784.
3. Morita H, Wu JS, Zipes DP. The QT syndromes: long and short. Lancet. 2008; 372: 750-763.
4. Priori SG, Napolitano C, Schwartz PJ. Low penetrance in the long-QT syndrome: clinical impact. Circulation. 1999; 99: 529-533.
5. Vincent GM. The molecular genetics of the long QT syndrome: genes causing fainting and sudden death. Annu Rev Med. 1998; 49: 263-274.
6. Ali RH, Zareba W, Moss AJ, Schwartz PJ, Benhorin J, Vincent GM, Locati EH, Priori S, Napolitano C, Towbin JA, Hall WJ, Robinson JL, Andrews ML, Zhang L, Timothy K, Medina A. Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with the long QT syndrome. Am J Cardiol. 2000; 85: 457-461.
7. Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C, Denjoy I, Guicheney P, Breithardt G, Keating MT, Towbin JA, Beggs AH, Brink P, Wilde AA, Toivonen L, Zareba W, Robinson JL, Timothy KW, Corfield V, Wattanasirichaigoon D, Corbett C, Haverkamp W, Schulze-Bahr E, Lehmann MH, Schwartz K, Coumel P, Bloise R. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation. 2001; 103: 89-95.
8. Viskin S, Rosovski U, Sands AJ, Chen E, Kistler PM, Kalman JM, Rodriguez Chavez L, Iturralde Torres P, Cruz FFE, Centurión OA, Fujiki A, Maury P, Chen X, Krahn AD, Roithinger F, Zhang L, Vincent GM, Zeltser D. Inaccurate electrocardiographic interpretation of long QT: the majority of physicians cannot recognize a long QT when they see one. Heart Rhythm. 2005; 2: 569-574.
9. Kaufman ES, Priori SG, Napolitano C, Schwartz PJ, Iyengar S, Elston RC, Schnell AH, Gorodeski EZ, Rammohan G, Bahhur NO, Connuck D, Verrilli L, Rosenbaum DS, Brown AM. Electrocardiographic prediction of abnormal genotype in congenital long QT syndrome: experience in 101 related family members. J Cardiovasc Electrophysiol. 2001; 12: 455-461.
10. Vincent GM, Timothy KW, Leppert M, Keating M. 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.
11. Ackerman MJ, Splawski I, Makielski JC, Tester DJ, Will ML, Timothy KW, Keating MT, Jones G, Chadha M, Burrow CR, Stephens JC, Xu C, Judson R, Curran ME. Spectrum and prevalence of cardiac sodium channel variants among black, white, Asian, and Hispanic individuals: implications for arrhythmogenic susceptibility and Brugada/long QT syndrome genetic testing. Heart Rhythm. 2004; 1: 600-607.
12. Walker BD, Krahn AD, Klein GJ, Skanes AC, Yee R, Wang J, Hegele RA. Effect of change in posture and exercise on repolarization in patients with long QT syndrome with HERG channel mutations. Can J Cardiol. 2005; 21: 33-38.
13. McLaughlin NB, Campbell RWF, Murray A. Accuracy of four automatic QT measurement techniques in cardiac patients and healthy subjects. Heart. 1996; 76: 422-426.
14. Bazett HC. An analysis of time relations of electrocardiograms. Heart. 1920; 7: 353-370.
15. Krahn AD, Klein GJ, Yee R. Hysteresis of the RT interval with exercise: a new marker for the long-QT syndrome? Circulation. 1997; 96: 1551-1556.
16. Takenaka K, Ai T, Shimizu W, Kobori A, Ninomiya T, Otani H, Kubota T, Takaki H, Kamakura S, Horie M. Exercise stress test amplifies genotype-phenotype correlation in the LQT1 and LQT2 forms of the long-QT syndrome. Circulation. 2003; 107: 838-844.
17. Shimizu W, Noda T, Takaki H, Kurita T, Nagaya N, Satomi K, Suyama K, Aihara N, Kamakura S, Sunagawa K, Echigo S, Nakamura K, Ohe T, Towbin JA, Napolitano C, Priori SG. Epinephrine unmasks latent mutation carriers with LQT1 form of congenital long-QT syndrome. J Am Coll Cardiol. 2003; 41: 633-642.
18. Shimizu W, Noda T, Takaki H, Nagaya N, Satomi K, Kurita T, Suyama K, Aihara N, Sunagawa K, Echigo S, Miyamoto Y, Yoshimasa Y, Nakamura K, Ohe T, Towbin JA, Priori SG, Kamakura S. Diagnostic value of epinephrine test for genotyping LQT1, LQT2, and LQT3 forms of congenital long QT syndrome. Heart Rhythm. 2004; 1: 276-283.
19. Ackerman MJ, Khositseth A, Tester DJ, Hejlik JB, Shen WK, Porter CB. Epinephrine-induced QT interval prolongation: a gene-specific paradoxical response in congenital long QT syndrome: Mayo Clin Proc. 2002; 77: 413-421.
20. Vyas H, Hejlik J, Ackerman MJ. Epinephrine QT stress testing in the evaluation of congenital long-QT syndrome: diagnostic accuracy of the paradoxical QT response. Circulation. 2006; 113: 1385-1392.
21. Swan H, Viitasalo M, Piippo K, Laitinen P, Kontula K, Toivonen L. Sinus node function and ventricular repolarization during exercise stress test in long QT syndrome patients with KvLQT1 and HERG potassium channel defects. J Am Coll Cardiol. 1999; 34: 823-829.
22. Krahn AD, Yee R, Chauhan V, Skanes AC, Wang J, Hegele RA, Klein GJ. Beta blockers normalize QT hysteresis in long QT syndrome. Am Heart J. 2002; 143: 528-534.