Korean Circulation Journal

The Korean Society of Cardiology (대한심장학회)
권호 표지
DOI QR코드

DOI QR Code

Management of Patients with Long QT Syndrome

  • Cho, Yongkeun (Department of Internal Medicine, Kyungpook National University Hospital)
  • Received : 2016.02.11
  • Accepted : 2016.03.22
  • Published : 2016.11.30
⟨ Previous Next ⟩

Abstract

Long QT syndrome (LQTS) is a rare cardiac channelopathy associated with syncope and sudden death due to torsades de pointes and ventricular fibrillation. Syncope and sudden death are frequently associated with physical and emotional stress. Management of patients with LQTS consists of life-style modification, ${\beta}$-blockers, left cardiac sympathetic denervation (LCSD), and implantable cardioverter-defibrillator (ICD) implantation. Prohibition of competitive exercise and avoidance of QT-prolonging drugs are important issues in life-style modification. Although ${\beta}$-blockers are the primary treatment modality for patients with LQTS, these drugs are not completely effective in some patients. Lifelong ICD implantation in young and active patients is associated with significant complications. LCSD is a relatively simple and highly effective surgical procedure. However, LCSD is rarely used.

Keywords

References

  1. Moss AJ, Schwartz PJ, Crampton RS, et al. The long QT syndrome. Prospective longitudinal study of 328 families. Circulation 1991;84:1136-44. https://doi.org/10.1161/01.CIR.84.3.1136
  2. Lee YS, Kwon BS, Kim GB, et al. Long QT syndrome: a Korean single center study. J Korean Med Sci 2013;28:1454-60. https://doi.org/10.3346/jkms.2013.28.10.1454
  3. Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm 2013;10:1932-63. https://doi.org/10.1016/j.hrthm.2013.05.014
  4. Schwartz PJ, Priori SG, Spazzolini C, et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for lifethreatening arrhythmias. Circulation 2001;103:89-95. https://doi.org/10.1161/01.CIR.103.1.89
  5. Choi G, Kopplin LJ, Tester DJ, Will ML, Haglund CM, Ackerman MJ. Spectrum and frequency of cardiac channel defects in swimmingtriggered arrhythmia syndromes. Circulation 2004;110:2119-24. https://doi.org/10.1161/01.CIR.0000144471.98080.CA
  6. Zipes DP, Ackerman MJ, Estes NA 3rd, Grant AO, Myerburg RJ, Van Hare G. Task Force 7: arrhythmias. J Am Coll Cardiol 2005;45:1354-63. https://doi.org/10.1016/j.jacc.2005.02.014
  7. Pelliccia A, Fagard R, Bjornstad HH, et al. Recommendations for competitive sports participation in athletes with cardiovascular disease: a consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology. Eur Heart J 2005;26:1422-45. https://doi.org/10.1093/eurheartj/ehi325
  8. Johnson JN, Ackerman MJ. Return to play? Athletes with congenital long QT syndrome. Br J Sports Med 2013;47:28-33. https://doi.org/10.1136/bjsports-2012-091751
  9. Ackerman MJ, Zipes DP, Kovacs RJ, Maron BJ. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 10: The Cardiac Channelopathies: A Scientific Statement From the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015;66:2424-8. https://doi.org/10.1016/j.jacc.2015.09.042
  10. Lampert R, Olshansky B, Heidbuchel H, et al. Safety of sports for athletes with implantable cardioverter-defibrillators: results of a prospective, multinational registry. Circulation 2013;127:2021-30. https://doi.org/10.1161/CIRCULATIONAHA.112.000447
  11. Christian S, Somerville M, Taylor S, Atallah J. Exercise and $\beta$-blocker therapy recommendations for inherited arrhythmogenic conditions. Cardiol Young 2016;26:1123-9. https://doi.org/10.1017/S1047951115001894
  12. Loar RW, Bos JM, Cannon BC, Ackerman MJ. Sudden cardiac arrest during sex in patients with either catecholaminergic polymorphic ventricular tachycardia or long-QT syndrome: a rare but shocking experience. J Cardiovasc Electrophysiol 2015;26:300-4. https://doi.org/10.1111/jce.12600
  13. 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;33:327-32. https://doi.org/10.1016/S0735-1097(98)00578-6
  14. Seth R, Moss AJ, McNitt S, et al. Long QT syndrome and pregnancy. J Am Coll Cardiol 2007;49:1092-8. https://doi.org/10.1016/j.jacc.2006.09.054
  15. Schwartz PJ, Ackerman MJ, George AL Jr, Wilde AA. Impact of genetics on the clinical management of channelopathies. J Am Coll Cardiol 2013;62:169-80. https://doi.org/10.1016/j.jacc.2013.04.044
  16. Rosero SZ, Zareba W, Moss AJ, et al. Asthma and the risk of cardiac events in the Long QT syndrome Investigative Group. Am J Cardiol 1999;84:1406-11. https://doi.org/10.1016/S0002-9149(99)00586-X
  17. Thottathil P, Acharya J, Moss AJ, et al. Risk of cardiac events in patients with asthma and long-QT syndrome treated with beta2 agonists. Am J Cardiol 2008;102:871-4. https://doi.org/10.1016/j.amjcard.2008.05.029
  18. Mall M, Wissner A, Schreiber R, et al. Role of K(V)LQT1 in cyclic adenosine monophosphate-mediated Cl(-) secretion in human airway epithelia. Am J Respir Cell Mol Biol 2000;23:283-9. https://doi.org/10.1165/ajrcmb.23.3.4060
  19. Collins S, Widger J, Davis A, Massie J. Management of asthma in children with long QT syndrome. Paediatr Respir Rev 2012;13:100-5.
  20. Crean AM, Abdel-Rahman SE, Greenwood JP. A sweet tooth as the root cause of cardiac arrest. Can J Cardiol 2009;25:e357-8. https://doi.org/10.1016/S0828-282X(09)70723-8
  21. Lin C, Ke X, Ranade V, Somberg J. The additive effects of the active component of grapefruit juice (naringenin) and antiarrhythmic drugs on HERG inhibition. Cardiology 2008;110:145-52. https://doi.org/10.1159/000111923
  22. Abu-Zeitone A, Peterson DR, Polonsky B, McNitt S, Moss AJ. Oral contraceptive use and the risk of cardiac events in patients with long QT syndrome. Heart Rhythm 2014;11:1170-5. https://doi.org/10.1016/j.hrthm.2014.04.016
  23. Zhang C, Kutyifa V, Moss AJ, McNitt S, Zareba W, Kaufman ES. Long-QT syndrome and therapy for attention deficit/hyperactivity disorder. J Cardiovasc Electrophysiol 2015;26:1039-44. https://doi.org/10.1111/jce.12739
  24. Moss AJ, Zareba W, Hall WJ, et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 2000;101:616-23. https://doi.org/10.1161/01.CIR.101.6.616
  25. Park YM, Kim SJ, Park CH, et al. Repeated aborted sudden cardiac death with long QT syndrome in a patient with anomalous origin of the right coronary artery from the left coronary cusp. Korean Circ J 2013:43:830-3. https://doi.org/10.4070/kcj.2013.43.12.830
  26. Hocini M, Pison L, Proclemer A, et al. Diagnosis and management of patients with inherited arrhythmia syndromes in Europe: results of the European Heart Rhythm Association Survey. Europace 2014;16:600-3. https://doi.org/10.1093/europace/euu074
  27. Vincent GM, Schwartz PJ, Denjoy I, et al. High efficacy of betablockers in long-QT syndrome type 1: contribution of noncompliance and QT-prolonging drugs to the occurrence of beta-blocker treatment "failures". Circulation 2009;119:215-21. https://doi.org/10.1161/CIRCULATIONAHA.108.772533
  28. Priori SG, Napolitano C, Schwartz PJ, et al. Association of long QT syndrome loci and cardiac events among patients treated with betablockers. JAMA 2004;292:1341-4. https://doi.org/10.1001/jama.292.11.1341
  29. Goldenberg I, Thottathil P, Lopes CM, et al. Trigger-specific ionchannel mechanisms, risk factors, and response to therapy in type 1 long QT syndrome. Heart Rhythm 2012;9:49-56. https://doi.org/10.1016/j.hrthm.2011.08.020
  30. Kim JA, Lopes CM, Moss AJ, et al. Trigger-specific risk factors and response to therapy in long QT syndrome type 2. Heart Rhythm 2010;7:1797-805. https://doi.org/10.1016/j.hrthm.2010.09.011
  31. Viskin S, Halkin A. Treating the long-QT syndrome in the era of implantable defibrillators. Circulation 2009;119:204-6. https://doi.org/10.1161/CIRCULATIONAHA.108.826198
  32. Koponen M, Marjamaa A, Hiippala A, et al. Follow-up of 316 molecularly defined pediatric long-QT syndrome patients: clinical course, treatments, and side effects. Circ Arrhythm Electrophysiol 2015;8:815-23. https://doi.org/10.1161/CIRCEP.114.002654
  33. Poterucha JT, Bos JM, Cannon BC, Ackerman MJ. Frequency and severity of hypoglycemia in children with beta-blocker-treated long QT syndrome. Heart Rhythm 2015;12:1815-9. https://doi.org/10.1016/j.hrthm.2015.04.034
  34. Chockalingam P, Crotti L, Girardengo G, et al. Not all beta-blockers are equal in the management of long QT syndrome types 1 and 2: Higher recurrence of events under metoprolol. J Am Coll Cardiol 2012;60:2092–9. https://doi.org/10.1016/j.jacc.2012.07.046
  35. Schwartz PJ, Ackerman MJ. The long QT syndrome: a transatlantic clinical approach to diagnosis and therapy. Eur Heart J 2013;34:3109-16. https://doi.org/10.1093/eurheartj/eht089
  36. Schwartz PJ. My Approach to the long QT syndrome(LQTS). Trends Cardiovasc Med 2015;25:376-7. https://doi.org/10.1016/j.tcm.2014.09.017
  37. Ackerman MJ. My Approach to treatment of the congenital long QT syndromes. Trends Cardiovasc Med 2015;25:67-9. https://doi.org/10.1016/j.tcm.2014.07.007
  38. Abu-Zeitone A, Peterson DR, Polonsky B, McNitt S, Moss AJ. Efficacy of different beta-blockers in the treatment of long QT syndrome. J Am Coll Cardiol 2014;64:1352-8. https://doi.org/10.1016/j.jacc.2014.05.068
  39. Schwartz PJ. Cutting nerves and saving lives. Heart Rhythm 2009;6:760-3. https://doi.org/10.1016/j.hrthm.2009.04.009
  40. Bos JM, Bos KM, Johnson JN, Moir C, Ackerman MJ. Left cardiac sympathetic denervation in long QT syndrome: analysis of therapeutic nonresponders. Circ Arrhythm Electrophysiol 2013;6:705-11. https://doi.org/10.1161/CIRCEP.113.000102
  41. Schwartz PJ, Priori SG, Cerrone M, et al. Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation 2004;109:1826-33. https://doi.org/10.1161/01.CIR.0000125523.14403.1E
  42. Schwartz PJ, Snebold NG, Brown AM. Effects of unilateral cardiac sympathetic denervation on the ventricular fibrillation threshold. Am J Cardiol 1976;37:1034-40. https://doi.org/10.1016/0002-9149(76)90420-3
  43. Schwartz PJ, Stone HL. Effects of unilateral stellectomy upon cardiac performance during exercise in dogs. Circ Res 1979;44:637-45. https://doi.org/10.1161/01.RES.44.5.637
  44. Antiel RM, Bos JM, Joyce DD, et al. Quality of life after videoscopic left cardiac sympathetic denervation in patients with potentially lifethreatening cardiac channelopathies/cardiomyopathies. Heart Rhythm 2016;13:62-9. https://doi.org/10.1016/j.hrthm.2015.09.001
  45. Schwartz PJ, Spazzolini C, Priori SG, et al. Who are the long-QT syndrome patients who receive an implantable cardioverterdefibrillator and what happens to them?: data from the European Long-QT Syndrome Implantable Cardioverter-Defibrillator (LQTS ICD) Registry. Circulation 2010;122:1272-82. https://doi.org/10.1161/CIRCULATIONAHA.110.950147
  46. Olde Nordkamp LR, Wilde AA, Tijssen JG, Knops RE, van Dessel PF, de Groot JR. The ICD for primary prevention in patients with inherited cardiac diseases: indications, use, and outcome: a comparison with secondary prevention. Circ Arrhythm Electrophysiol 2013;6:91-100. https://doi.org/10.1161/CIRCEP.112.975268
  47. Monnig G, Kobe J, Loher A, et al. Role of implantable cardioverter defibrillator therapy in patients with acquired long QT syndrome: a long-term follow-up. Europace 2012;14:396-401. https://doi.org/10.1093/europace/eur316
  48. Gaba P, Bos JM, Cannon BC, et al. Implantable cardioverterdefibrillator explantation for overdiagnosed or overtreated congenital long QT syndrome. Heart Rhythm 2016;13:879-85. https://doi.org/10.1016/j.hrthm.2015.12.008
  49. Olde Nordkamp LR, Postema PG, Knops RE, et al. Implantable cardioverter-defibrillator harm in young patients with inherited arrhythmia syndromes: a systematic review and meta-analysis of inappropriate shocks and complications. Heart Rhythm 2016;13:443-54. https://doi.org/10.1016/j.hrthm.2015.09.010
  50. Horner JM, Kinoshita M, Webster TL, Haglund CM, Friedman PA, Ackerman MJ.,Implantable cardioverter defibrillator therapy for congenital long QT syndrome: a single-center experience. Heart Rhythm 2010;7:1616-22. https://doi.org/10.1016/j.hrthm.2010.08.023
  51. Compton SJ, Lux RL, Ramsey MR, et al. Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium. Circulation 1996;94:1018-22. https://doi.org/10.1161/01.CIR.94.5.1018
  52. Etheridge SP, Compton SJ, Tristani-Firouzi M, Mason JW. A new oral therapy for long QT syndrome: long-term oral potassium improves repolarization in patients with HERG mutations. J Am Coll Cardiol 2003;42:1777-82. https://doi.org/10.1016/j.jacc.2003.07.006

Cited by

  1. Trends in the use of implantable cardioverter‐defibrillators for prevention of sudden cardiac arrest: A South Korean nationwide population‐based study vol.42, pp.8, 2019, https://doi.org/10.1111/pace.13741
  2. Polyunsaturated fatty acids produce a range of activators for heterogeneous I Ks channel dysfunction vol.152, pp.2, 2016, https://doi.org/10.1085/jgp.201912396
  3. Surgical Approach in Congenital Long QT Interval Syndrome Patients vol.18, pp.1, 2020, https://doi.org/10.2478/chilat-2020-0016
  4. Long QT Syndrome and Electrical Storm: Is Implanted Cardiac Defibrillator the Final Destination in Long QT Syndrome Management? vol.62, pp.5, 2020, https://doi.org/10.33678/cor.2020.076
  5. Video-thoracoscopic left cardiac sympathetic denervation for long-QT syndrome vol.29, pp.3, 2016, https://doi.org/10.1177/0218492320971492
  6. Long QT syndrome type 2: mechanism-based therapies vol.17, pp.8, 2016, https://doi.org/10.2217/fca-2020-0234