Conclusions
There is a high and in many cases inappropriate use of ICDs in patients with long QT syndrome with syncope. The pros and cons of the ICD and of the other available therapies should be clearly explained to all patients before recommending one or another.
It should be remembered that LQTS is profoundly different from most of the more prevalent conditions for which an ICD is usually recommended, and that among the special features of LQTS the tight relationship between release of catecholamines and life-threatening arrhythmias is an important one capable of initiating storms of shocks.
The wise use of the ICD in LQTS can save many lives. The unwise use of the ICD in LQTS can ruin many lives. A competent and caring physician should not be unmindful of his/her complex responsibilities toward the - usually young - LQTS patients.
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References
Schwartz PJ, Periti M, Malliani A (1975) The long Q-T syndrome. Am Heart J 89:378–390
Schwartz PJ (1985) Idiopathic long QT syndrome: progress and questions. Am Heart J 109:399–411
Schwartz PJ, Priori SG, Napolitano C (2000) The long QT syndrome. In: Zipes DP, Jalife J (eds) Cardiac electrophysiology. From cell to bedside, 3rd edn. Saunders, Philadelphia, pp 597–615
Schwartz PJ (2005) Management of the long QT syndrome. Nat Clin Pract Cardiovasc Med 2:346–351
Priori SG, Schwartz PJ, Napolitano C et al (2003) Risk stratification in the long-QT syndrome. N Engl J Med 348:1866–1874
Schwartz PJ, Priori SG (2004) Long QT syndrome: genotype-phenotype correlations. In: Zipes DP, Jalife J (eds) Cardiac electrophysiology. From cell to bedside, 3rd edn. Saunders, Philadelphia, pp 651–659
Moss AJ, Zareba W, Benhorin J et al (1995) ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation 92:2929–2934
Zhang L, Timothy KW, Vincent GM et al (2000) Spectrum of ST-T-wave patterns and repolarization parameters in congenital long QT syndrome. ECG findings identify genotypes. Circulation 102:2849–2855
Schwartz PJ, Priori SG, Spazzolini C et al (2001) Genotype-phenotype correlation in the long QT syndrome. Gene-specific triggers for life-threatening arrhythmias. Circulation 103:89–95
Splawski I, Shen J, Timothy KW et al (2000) Spectrum of mutations in long-QT syndrome genes. Circulation 102:1178–1185
Schwartz PJ (1980) The long QT syndrome. In: Kulbertus HE, Wellens HJJ (eds) Sudden death. M Nijhoff, The Hague, pp 358–378
Priori SG, Napolitano C, Schwartz PJ (1999) Low penetrance in the long QT syndrome. Clinical impact. Circulation 99:529–533
Moss AJ, Schwartz PJ (2005) 25th Anniversary of the international Long QT Syndrome Registry: an ongoing quest to uncover the secrets of LQTS. Circulation 111:1199–1201
Moss AJ, Zareba W, Hall WJ et al (2000) Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 101:616–623
Vincent GM, Bithell C, Schwartz PJ et al (2003) Efficacy of beta-blockers in the LQT1 genotype of long QT syndrome. Circulation 108:IV–506 (abs)
Priori SG, Napolitano C, Schwartz PJ et al (2004) Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers. JAMA 292:1341–1344
Schwartz PJ, Priori SG, Cerrone M et al (2004) Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation 109:1826–1833
Schwartz PJ, Priori SG, Locati EH et al (1995) Long QT syndrome patients with mutations on the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate. Implications for gene-specific therapy. Circulation 92:3381–3386
Zareba W, Moss AJ, Daubert JP et al (2003) Implantable cardioverter defibrillator in high-risk long QT syndrome patients. J Cardiovasc Electrophysiol 14:337–341
Crotti L, Spazzolini C, De Ferrari GM et al (2004) Is the implantable defibrillator appropriately used in the long QT syndrome? Data from the European Registry. Heart Rhythm 1(Suppl):582
Mönig G, Köe J, Löer A et al (2005) Implantable cardioverter-defibrillator therapy in patients with congenital long-QT syndrome: a long-term follow-up. Heart Rhythm 2:497–504
Kimbrough J, Moss AJ, Zareba W et al (2001) Clinical implications for affected parents and siblings of probands with long QT syndrome. Circulation 104:557–562
Viskin S (2003) Implantable cardioverter defibrillator in high-risk long QT syndrome patients. J Cardiovasc Electrophysiol 14:1130–1131
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Schwartz, P.J., Spazzolini, C., Crotti, L. (2006). ICD for the Long QT Syndrome: Which Indications, Complications, and Results?. In: Raviele, A. (eds) Cardiac Arrhythmias 2005. Springer, Milano. https://doi.org/10.1007/88-470-0371-7_43
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