Lifestyle modification and medical treatment of long QT syndrome

Authors

  • Omar Rezk Alshaer Department of Internal Medicine, Security Forces Hospital, Riyadh, Saudi Arabia
  • Abdullah Obaid Binobaid Department of Internal Medicine, Security Forces Hospital, Riyadh, Saudi Arabia
  • Saeed Dawas Alwadai College of Medicine, Najran University, Najran, Saudi Arabia
  • Wejdan Hani Alhakeem College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia
  • Khalid Abdullah Al-Attas Security Forces Hospital, Arar, Saudi Arabia
  • Reenad Ahmed Takrouni College of Medicine, Batterjee Medical College, Jeddah, Saudi Arabia
  • Hameed Mahdi Maki College of Medicine, Xi’an Jiaotong University, Xi’an, China
  • Yasir Ali Al Murdhimah College of Medicine, Najran University, Najran, Saudi Arabia
  • Ali Mohammed Aleid College of Medicine, Medical University of Lodz, Lodz, Poland
  • Jowhara Salem Badyan College of Medicine, Taif University, Taif, Saudi Arabia
  • Raghad Faisal Al Harthi College of Medicine, Taif University, Taif, Saudi Arabia

DOI:

https://doi.org/10.18203/2394-6040.ijcmph20213578

Keywords:

Cardiac, Treatment, LQTS, Lifestyle, Pharmacological

Abstract

Studies have shown that many manifestations can be observed for patients suffering from long QT syndrome (LQTS), as it is a common cause of syncope and mortality among younger patients, in addition to convulsions. Many management modalities for LQTS have been described in the literature including medical treatment modalities and lifestyle modification approaches. However, evidence regarding the outcomes of these approaches is continuously updating. In this study, we have reviewed findings from the current studies in the literature about the medical treatment and lifestyle modifications for patients suffering from LQTS. Furthermore, studies have shown that β-blockers are effective modalities and should be used at a maximum dose. However, the potential side events should be considered and adequately managed and patient compliance should be maintained along the course of treatment. Implantable cardioverter-defibrillator (ICD) implantation and LCSD were also discussed in the present study with their favorable indications. Additionally, lifestyle modifications were also important and have been reported with favorable events and therefore, these should be considered in such situations. However, evidence regarding some approaches as limiting competitive exercises is still conflicting, indicating the need for future investigations.

References

Schwartz PJ. 1970-2020: 50 years of research on the long QT syndrome-from almost zero knowledge to precision medicine. Eur Heart J. 2021;42(11):1063-72.

Schwartz PJ, Ackerman MJ. The long QT syndrome: a transatlantic clinical approach to diagnosis and therapy. Eur Heart J. 2013;34(40):3109-16.

Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013;10(12):1932-63.

Moss AJ, Schwartz PJ, Crampton RS, Tzivoni D, Locati EH, MacCluer J, et al. The long QT syndrome. Prospective longitudinal study of 328 families. Circulation. 1991;84(3):1136-44.

Lee YS, Kwon BS, Kim GB, Oh S, Bae E, Park S, et al. Long QT syndrome: a Korean single center study. J Korean Med Sci. 2013;28(10):1454-60.

Schwartz PJ, Periti M, Malliani A. The long Q-T syndrome. Am Heart J. 1975;89(3):378-90.

Garson A, Dick M, Fournier A, Gillette PC, Hamilton R, Kugler JD, et al. The long QT syndrome in children. An international study of 287 patients. Circulation. 1993;87(6):1866-72.

Ghozy S, Tran L, Naveed S, Quynh TTH, Zayan AH, Waqas A, et al. Association of breastfeeding status with risk of autism spectrum disorder: a systematic review, dose-response analysis and meta-analysis. Asian J Psychiatr. 2020;48:101916.

Mahmoud AR, Dahy A, Dibas M, Abbas AS, Ghozy S, El-Qushayri AE. Association between sarcoidosis and cardiovascular comorbidity: a systematic review and meta-analysis. Heart Lung. 2020;49(5):512-7.

Ghozy S, Nam NH, Radwan I, Karimzadeh S, Tieu TM, Hashan MR, et al. Therapeutic efficacy of hepatitis B virus vaccine in treatment of chronic HBV infections: a systematic review and meta-analysis. Rev Med Virol. 2020;30(3):2089.

Hashan MR, Ghozy S, El-Qushayri AE, Pial RH, Hossain MA, AlKibria GM. Association of dengue disease severity and blood group: a systematic review and meta-analysis. Rev Med Virol. 2021;31(1):1-9.

Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, Benhorin J, et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation. 2000;101(6):616-23.

Park YM, Kim SJ, Park CH, Kang WC, Shin M, Koh KK, 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(12):830-3.

Hocini M, Pison L, Proclemer A, Larsen TB, Madrid A, Blomström-Lundqvist C. Diagnosis and management of patients with inherited arrhythmia syndromes in Europe. Europ Soc Cardiol. 2014;16(4):600-3.

Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C, et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation. 2001;103(1):89-95.

Priori SG, Napolitano C, Schwartz PJ, Grillo M, Bloise R, Ronchetti E, et al. Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers. JAMA. 2004;292(11):1341-4.

Vincent GM, Schwartz PJ, Denjoy I, Swan H, Bithell C, Spazzolini C, et al. High efficacy of beta-blockers in long-QT syndrome type 1: contribution of noncompliance and QT-prolonging drugs to the occurrence of beta-blocker treatment "failures". Circulation. 2009;119(2):215-21.

Goldenberg I, Thottathil P, Lopes CM, Moss AJ, McNitt S, O-Uchi J, et al. Trigger-specific ion-channel mechanisms, risk factors, and response to therapy in type 1 long QT syndrome. Heart Rhythm. 2012;9(1):49-56.

Kim JA, Lopes CM, Moss AJ, McNitt S, Barsheshet A, Robinson JL, et al. Trigger-specific risk factors and response to therapy in long QT syndrome type 2. Heart Rhythm. 2010;7(12):1797-805.

Seth R, Moss AJ, McNitt S, Zareba W, Andrews ML, Qi M, et al. Long QT syndrome and pregnancy. J Am Coll Cardiol. 2007;49(10):1092-8.

Koponen M, Marjamaa A, Hiippala A, Happonen J, Havulinna AS, Salomaa V, et al. Follow-up of 316 molecularly defined pediatric long-QT syndrome patients: clinical course, treatments, and side effects. Circulation Arrhythmia Electrophysiol. 2015;8(4):815-23.

Viskin S, Halkin A. Treating the long-QT syndrome in the era of implantable defibrillators. Circulation. 2009;119(2):204-6.

Chockalingam P, Crotti L, Girardengo G, Johnson JN, Harris KM, Heijden JFVD, 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(20):2092-9.

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(13):1352-8.

Schwartz PJ, Spazzolini C, Priori SG, Crotti L, Vicentini A, Landolina M, et al. Who are the long-QT syndrome patients who receive an implantable cardioverter-defibrillator and what happens to them?: data from the European long-QT syndrome implantable cardioverter-defibrillator (LQTS ICD) registry. Circulation. 2010;122(13):1272-82.

Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013;10(12):1932-63.

Mönnig G, Köbe J, Löher A, Wasmer K, Milberg P, Zellerhoff S, et al. Role of implantable cardioverter defibrillator therapy in patients with acquired long QT syndrome: a long-term follow-up. Europace. 2012;14(3):396-401.

Gaba P, Bos JM, Cannon BC. Implantable cardioverter-defibrillator explantation for overdiagnosed or overtreated congenital long QT syndrome. Heart Rhythm. 2016;13(4):879-85.

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(11):1616-22.

Nordkamp LRO, 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(2):443-54.

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(4):705-11.

Schwartz PJ, Priori SG, Cerrone M, Spazzolini C, Odero A, Napolitano C, et al. Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation. 2004;109(15):1826-33.

Ackerman MJ. MY APPROACH to treatment of the congenital long QT syndromes. Trends Cardiovasc Med. 2015;25(1):67-9.

Schwartz PJ, Snebold NG, Brown AM. Effects of unilateral cardiac sympathetic denervation on the ventricular fibrillation threshold. Am J Cardiol. 1976;37(7):1034-40.

Cho Y. Management of patients with long QT syndrome. Korean Circ J. 2016;46(6):747-52.

Compton SJ, Lux RL, Ramsey MR, Strelich KR, Sanguinetti MC, Green LS, et al. Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium. Circulation. 1996;94(5):1018-22.

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(10):1777-82.

Zipes DP, Ackerman MJ, Estes NA, Grant AO, Myerburg RJ, Hare GV. Task force 7: arrhythmias. J Am Coll Cardiol. 2005;45(8):1354-63.

Pelliccia A, Fagard R, Bjørnstad HH, Anastassakis A, Arbustini E, Assanelli D, 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(14):1422-45.

Johnson JN, Ackerman MJ. Return to play? Athletes with congenital long QT syndrome. Br J Sports Med. 2013;47(1):28-33.

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(20):2021-30.

Christian S, Somerville M, Taylor S, Atallah J. Exercise and β-blocker therapy recommendations for inherited arrhythmogenic conditions. Cardiology Young. 2016;26(6):1123-9.

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(3):300-4.

Wilde AA, Jongbloed RJ, Doevendans PA, Düren DR, Hauer RN, Langen IMV, 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(2):327-32.

Schwartz PJ, Ackerman MJ, George AL, Wilde AAM. Impact of genetics on the clinical management of channelopathies. J Am Coll Cardiol. 2013;62(3):169-80.

Rosero SZ, Zareba W, Moss AJ, Robinson JL, Ali RHH, Locati EH, et al. Asthma and the risk of cardiac events in the long QT syndrome. Long QT syndrome investigative group. Am J Cardiol. 1999;84(12):1406-11.

Thottathil P, Acharya J, Moss AJ, Jons C, McNitt S, Goldenberg I, et al. Risk of cardiac events in patients with asthma and long-QT syndrome treated with beta(2) agonists. Am J Cardiol. 2008;102(7):871-4.

Mall M, Wissner A, Schreiber R, Kuehr J, Seydewitz HH, Brandis M, 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(3):283-9.

Collins S, Widger J, Davis A, Massie J. Management of asthma in children with long QT syndrome. Paediatr Respirat Rev. 2012;13(2):100-5.

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(10):1039-44.

Wilde AAM, Amin AS, Postema PG. Diagnosis, management and therapeutic strategies for congenital long QT syndrome. Heart. 2021;2020:318259.

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Published

2021-08-27

How to Cite

Alshaer, O. R., Binobaid, A. O., Alwadai, S. D., Alhakeem, W. H., Al-Attas, K. A., Takrouni, R. A., Maki, H. M., Al Murdhimah, Y. A., Aleid, A. M., Badyan, J. S., & Al Harthi, R. F. (2021). Lifestyle modification and medical treatment of long QT syndrome. International Journal Of Community Medicine And Public Health, 8(9), 4637–4643. https://doi.org/10.18203/2394-6040.ijcmph20213578

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Section

Review Articles