Abstract
The practice of a sport doctor does not have many slippery steps. One, to be navigated carefully, concerns the assessment of the QT interval in a subject requesting a sport eligibility certificate. The two possible errors, either not realizing that it is prolonged or incorrectly assuming that it is, could have major consequences. Indeed, sudden death could be the price of the first error, and a major adverse effect on the quality of life could be the price of the second error.
In this chapter, we review the key features of the long QT syndrome (LQTS) and provide suggestions on how to avoid traditional errors. We show examples of potentially tragic oversights and then discuss a completely novel issue, representing a potential diagnostic trap, namely, the new evidence that young athletes sometimes present ECG abnormalities typical of LQTS but are fully reversible upon detraining and seem to constitute an “exercise-induced LQTS.” We conclude by sharing our views and practical suggestions.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Schwartz PJ, Ackerman MJ. The long QT syndrome: a transatlantic clinical approach to diagnosis and therapy. Eur Heart J. 2013;34:3109–16.
Schwartz PJ, Ackerman MJ, Antzelevitch C, Bezzina CR, Borggrefe M, Cuneo BF, Wilde AAM. Inherited cardiac arrhythmias. Nat Rev Dis Prim. 2020;6:58.
Schwartz PJ, Stramba-Badiale M, Crotti L, Pedrazzini M, Besana A, Bosi G, et al. Prevalence of the congenital long-QT syndrome. Circulation. 2009;120:1761–7.
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;109:1826–33.
Schwartz PJ. Cardiac sympathetic denervation to prevent life-threatening arrhythmias. Nat Rev Cardiol. 2014;11:346–53.
Dusi V, Pugliese L, De Ferrari GM, Odero A, Crotti L, Dagradi F, Castelletti S, Vicentini A, Rordorf R, Li C, Shkolnikova M, Spazzolini C, Schwartz PJ. Left cardiac sympathetic denervation for the long QT syndrome: An almost 50-Year single center experience provides novel guidance for management. JACC Clin Electrophysiol. 2021 (In press).
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;103:89–95.
Schwartz PJ, Ackerman MJ, George AL Jr, Wilde AAM. Impact of genetics on the clinical management of channelopathies. J Am Coll Cardiol. 2013;62:169–80.
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;122:1272–82.
Dagradi F, Spazzolini C, Castelletti S, Pedrazzini M, Kotta MC, Crotti L, Schwartz PJ. Exercise training-induced repolarization abnormalities masquerading as congenital long QT syndrome. Circulation. 2020;142:2405–15. https://doi.org/10.1161/CIRCULATIONAHA.120.048916.
Roden DM. Drug-induced prolongation of the QT interval. N Engl J Med. 2004;350:1013–22.
Schwartz PJ, Woosley RL. Predicting the unpredictable: drug-induced QT prolongation and Torsades de Pointes. J Am Coll Cardiol. 2016;67:1639–50.
Peyronnet R, Nerbonne JM, Kohl P. Cardiac mechano-gated ion channels and arrhythmias. Circ Res. 2016;118:311–29.
Schwartz PJ. Long QT syndrome and sport: my view. In: Thompson PD, Fernandez AB, editors. Exercise and sport cardiology, Exercise risks, cardiac arrhythmias and unusual problems in athletes, vol. 3. London: World Scientific Publishing Europe Ltd; 2018. p. 269–78. isbn:9781786342607.
Heidbuchel H, Arbelo E, D'Ascenzi F, Borjesson M, Boveda S, Castelletti S, et al. Recommendations for participation in leisure-time physical activity and competitive sports of patients with arrhythmias and potentially arrhythmogenic conditions. Part 2: ventricular arrhythmias, channelopathies, and implantable defibrillators. Europace. 2021;23:147–8. https://doi.org/10.1093/europace/euaa106.
Schwartz PJ, Vanoli E, Crotti L, Spazzolini C, Ferrandi C, Goosen A, et al. Neural control of heart rate is an arrhythmia risk modifier in long QT syndrome. J Am Coll Cardiol. 2008;51:920–9.
Crotti L, Spazzolini C, Porretta AP, Dagradi F, Taravelli E, Petracci B, et al. Vagal reflexes following an exercise stress test: a simple clinical tool for gene-specific risk stratification in the long QT syndrome. J Am Coll Cardiol. 2012;60:2515–24.
Schwartz PJ, Crotti L. QTc behavior during exercise and genetic testing for the long-QT syndrome. Circulation. 2011;124:2181–4.
Schwartz PJ. 1970-2020: 50 years of research on the long QT syndrome. From almost zero knowledge to precision medicine. Eur Heart J. 2021;42:1063–72. https://doi.org/10.1093/eurheartj/ehaa769.
Acknowledgments
The authors acknowledge the support of the Italian Ministry of Health grant RF-2016-02361501 “Does exercise training mostly unmask congenital long QT syndrome or simply reveals a novel form of acquired long QT syndrome in genetically predisposed youngsters practicing sports? Ethical and medical implications of a potentially dangerous misdiagnosis,” of grant ERA-CVD JTC-2018-026 “Electromechanical presages of sudden cardiac death in the young: integrating imaging, modeling, and genetics for patient stratification,” and of Leducq Foundation for Cardiovascular Research grant 18CVD05 “Towards Precision Medicine with Human iPSCs for Cardiac Channelopathies.”
The authors are grateful to Pinuccia De Tomasi for expert editorial support.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2022 The Author(s), under exclusive license to Springer Nature Switzerland AG
About this chapter
Cite this chapter
Schwartz, P.J., Castelletti, S., Dagradi, F. (2022). Fear of Sudden Death During Sport Activity and the Long QT Syndrome. In: Delise, P., Zeppilli, P. (eds) Sport-related sudden cardiac death. Springer, Cham. https://doi.org/10.1007/978-3-030-80447-3_9
Download citation
DOI: https://doi.org/10.1007/978-3-030-80447-3_9
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-80446-6
Online ISBN: 978-3-030-80447-3
eBook Packages: MedicineMedicine (R0)