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(Circulation. 2000;101:616.)
© 2000 American Heart Association, Inc.

Clinical Investigation and Reports

Effectiveness and Limitations of ß-Blocker Therapy in Congenital Long-QT Syndrome

Arthur J. Moss, MD; Wojciech Zareba, MD, PhD; W. Jackson Hall, PhD; Peter J. Schwartz, MD; Richard S. Crampton, MD; Jesaia Benhorin, MD; G. Michael Vincent, MD; Emanuela H. Locati, MD, PhD; Silvia G. Priori, MD, PhD; Carlo Napolitano, MD; Aharon Medina, MD; Li Zhang, MD; Jennifer L. Robinson, MS; Katherine Timothy, BA; Jeffrey A. Towbin, MD; Mark L. Andrews, BBA

From the Departments of Medicine (A.J.M., W.Z.), Biostatistics (W.J.H.), and Community and Preventive Medicine (J.L.R., M.L.A.), University of Rochester School of Medicine and Dentistry, Rochester, NY; the Department of Medicine (R.S.C.), University of Virginia Health Sciences Center, Charlottesville, Va; the Department of Cardiology (P.J.S.), Policlinico San Matteo IRCCS and University of Pavia; Molecular Cardiology and Electrophysiology Laboratory (S.G.P., C.N.), Fondazione S. Maugeri IRCCS, Pavia, Italy; the Department of Medicine (G.M.V., L.Z., K.T.), University of Utah School of Medicine, Salt Lake City, Utah; the Heiden Department of Cardiology (J.B., A.M.), Bikur Cholim Hospital, Hebrew University, Jerusalem, Israel; the Department of Pediatric Cardiology (J.A.T.), Baylor College of Medicine, Houston, Tex; and the Section of Cardiology (E.H.L.), Department of Clinical and Experimental Medicine, Universita Degli Studi Di Perugia, Perugia, Italy.

Correspondence to Arthur J. Moss, MD, Heart Research Follow-up Program, Box 653, University of Rochester Medical Center, Rochester, NY 14642. E-mail heartajm{at}heart.rochester.edu

Background—ß-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness and limitations of ß-blockers in this disorder have not been evaluated.

Methods and Results—The study population comprised 869 LQTS patients treated with ß-blockers. Effectiveness of ß-blockers was analyzed during matched periods before and after starting ß-blocker therapy, and by survivorship methods to determine factors associated with cardiac events while on prescribed ß-blockers. After initiation of ß-blockers, there was a significant (P<0.001) reduction in the rate of cardiac events in probands (0.97±1.42 to 0.31±0.86 events per year) and in affected family members (0.26±0.84 to 0.15±0.69 events per year) during 5-year matched periods. On-therapy survivorship analyses revealed that patients with cardiac symptoms before ß-blockers (n=598) had a hazard ratio of 5.8 (95% CI, 3.7 to 9.1) for recurrent cardiac events (syncope, aborted cardiac arrest, or death) during ß-blocker therapy compared with asymptomatic patients; 32% of these symptomatic patients will have another cardiac event within 5 years while on prescribed ß-blockers. Patients with a history of aborted cardiac arrest before starting ß-blockers (n=113) had a hazard ratio of 12.9 (95% CI, 4.7 to 35.5) for aborted cardiac arrest or death while on prescribed ß-blockers compared with asymptomatic patients; 14% of these patients will have another arrest (aborted or fatal) within 5 years on ß-blockers.

Conclusions—ß-blockers are associated with a significant reduction in cardiac events in LQTS patients. However, syncope, aborted cardiac arrest, and LQTS-related death continue to occur while patients are on prescribed ß-blockers, particularly in those who were symptomatic before starting this therapy.

Key Words: arrhythmia • syncope • death, sudden • heart arrest • long-QT syndrome

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				I. Splawski, K. W. Timothy, M. Tateyama, C. E. Clancy, A. Malhotra, A. H. Beggs, F. P. Cappuccio, G. A. Sagnella, R. S. Kass, and M. T. Keating Variant of SCN5A Sodium Channel Implicated in Risk of Cardiac Arrhythmia Science, August 23, 2002; 297(5585): 1333 - 1336. [Abstract] [Full Text] [PDF]

				M. Chinushi, H. Kasai, M. Tagawa, T. Washizuka, Y. Hosaka, Y. Chinushi, and Y. Aizawa Triggers of ventricular tachyarrhythmias and therapeutic effects of nicorandil in canine models of LQT2 and LQT3 syndromes J. Am. Coll. Cardiol., August 7, 2002; 40(3): 555 - 562. [Abstract] [Full Text] [PDF]

				C. Antzelevitch Sympathetic modulation of the long QT syndrome Eur. Heart J., August 2, 2002; 23(16): 1246 - 1252. [PDF]

				W. Shimizu, Y. Tanabe, T. Aiba, M. Inagaki, T. Kurita, K. Suyama, N. Nagaya, A. Taguchi, N. Aihara, K. Sunagawa, et al. Differential effects of beta-blockade on dispersion of repolarization in the absence and presence of sympathetic stimulation between the lqt1 and lqt2 forms of congenital long qt syndrome J. Am. Coll. Cardiol., June 19, 2002; 39(12): 1984 - 1991. [Abstract] [Full Text] [PDF]

				T. Nagatomo, C. T. January, B. Ye, H. Abe, Y. Nakashima, and J. C. Makielski Rate-dependent QT shortening mechanism for the LQT3 {Delta}KPQ mutant Cardiovasc Res, June 1, 2002; 54(3): 624 - 629. [Abstract] [Full Text] [PDF]

				M. J. Ackerman, A. Khositseth, D. J. Tester, J. B. Hejlik, W.-K. Shen, and C.-b. J. Porter Epinephrine-Induced QT Interval Prolongation: A Gene-Specific Paradoxical Response in Congenital Long QT Syndrome Mayo Clin. Proc., May 1, 2002; 77(5): 413 - 421. [Abstract] [PDF]

				S. G. Priori, C. Napolitano, M. Gasparini, C. Pappone, P. D. Bella, U. Giordano, R. Bloise, C. Giustetto, R. De Nardis, M. Grillo, et al. Natural History of Brugada Syndrome: Insights for Risk Stratification and Management Circulation, March 19, 2002; 105(11): 1342 - 1347. [Abstract] [Full Text] [PDF]

				R. Chatrath, C.-b. J. Porter, and M. J. Ackerman Role of Transvenous Implantable Cardioverter-Defibrillators in Preventing Sudden Cardiac Death in Children, Adolescents, and Young Adults Mayo Clin. Proc., March 1, 2002; 77(3): 226 - 231. [Abstract] [PDF]

				S. G. Priori, E. Aliot, C. Blomstrom-Lundqvist, L. Bossaert, G. Breithardt, P. Brugada, J. A. Camm, R. Cappato, S. M. Cobbe, C. Di Mario, et al. TASK FORCE ON SUDDEN CARDIAC DEATH, EUROPEAN SOCIETY OF CARDIOLOGY: Summary of Recommendations Europace, January 1, 2002; 4(1): 3 - 18. [Abstract] [PDF]

				M. J. Ackerman, B. L. Siu, W. Q. Sturner, D. J. Tester, C. R. Valdivia, J. C. Makielski, and J. A. Towbin Postmortem Molecular Analysis of SCN5A Defects in Sudden Infant Death Syndrome JAMA, November 14, 2001; 286(18): 2264 - 2269. [Abstract] [Full Text] [PDF]

				B Campanelli and J-M Chaudron Long term follow up of long QT syndrome treated by overdrive pacing Heart, November 1, 2001; 86(5): e14 - 14. [Abstract] [Full Text] [PDF]

				D. V. Exner, G. J. Klein, and E. N. Prystowsky Primary Prevention of Sudden Death With Implantable Defibrillator Therapy in Patients With Cardiac Disease: Can We Afford to Do It? (Can We Afford Not To?) Circulation, September 25, 2001; 104(13): 1564 - 1570. [Full Text] [PDF]

				P. Coumel and A. A.M. Wilde Learning From Mistakes: The Case of Clinical Electrophysiology: A Perspective on Evidence-Based Rhythmology Circulation, August 14, 2001; 104(7): 845 - 847. [Full Text] [PDF]

				S.G. Priori, E. Aliot, C. Blomstrom-Lundqvist, L. Bossaert, G. Breithardt, P. Brugada, A.J. Camm, R. Cappato, S.M. Cobbe, C. Di Mario, et al. Task Force on Sudden Cardiac Death of the European Society of Cardiology Eur. Heart J., August 2, 2001; 22(16): 1374 - 1450. [PDF]

				J. Kimbrough, A. J. Moss, W. Zareba, J. L. Robinson, W. J. Hall, J. Benhorin, E. H. Locati, A. Medina, C. Napolitano, S. Priori, et al. Clinical Implications for Affected Parents and Siblings of Probands With Long-QT Syndrome Circulation, July 31, 2001; 104(5): 557 - 562. [Abstract] [Full Text] [PDF]

				R.N.W. Hauer, E. Aliot, M. Block, A. Capucci, B. Luderitz, M. Santini, and P.E. Vardas Indications for implantable cardioverter defibrillator (ICD) therapy. Study Group on Guidelines on ICDs of the Working Group on Arrhythmias and the Working Group on Cardiac Pacing of the European Society of Cardiology Eur. Heart J., July 1, 2001; 22(13): 1074 - 1081. [PDF]

				J. A. Towbin, Z. Wang, and H. Li Genotype and Severity of Long QT Syndrome Drug Metab. Dispos., April 1, 2001; 29(4): 574 - 579. [Abstract] [Full Text]