open access

Vol 76, No 12 (2018)
Original articles
Published online: 2018-08-17
Submitted: 2018-03-12
Accepted: 2018-08-16
Get Citation

Implantable cardioverter-defibrillators in patients with long QT syndrome: a multicentre study

Agnieszka Zienciuk-Krajka, Maciej Sterliński, Artur Filipecki, Radosław Owczuk, Jacek Bednarek, Maciej Kempa, Sławomir Sielski, Marcin Dziduszko, Przemysław Mitkowski, Jarosław Kaźmierczak, Jacek Kuśnierz, Dariusz Michałkiewicz, Sebastian M. Stec, Marcin Gułaj, Ryszard Majcherek, Andrzej Lubiński, Grzegorz Raczak
DOI: 10.5603/KP.a2018.0177
·
Pubmed: 30251242
·
Kardiol Pol 2018;76(12):1687-1696.

open access

Vol 76, No 12 (2018)
Original articles
Published online: 2018-08-17
Submitted: 2018-03-12
Accepted: 2018-08-16

Abstract

Background: Implantable cardioverter-defibrillator (ICD) therapy has been proven effective in the prevention of sudden cardiac death, but data on outcomes of ICD therapy in the young and otherwise healthy patients with long QT syndrome (LQTS) are limited.

Aim: We sought to collect data on appropriate and inappropriate ICD discharges, risk factors, and ICD-related complications.

Methods: All LQTS patients implanted with an ICD in 14 centres were investigated. Demographic, clinical, and ICD therapy data were collected.

Results: The study included 67 patients (88% female). Median age at ICD implantation was 31 years (12–77 years). ICD indication was based on resuscitated cardiac arrest in 46 patients, syncope in 18 patients, and malignant family history in three patients. During a median follow-up of 48 months, 39 (58%) patients received one or more ICD therapies. Time to first appropriate discharge was up to 55 months. Inappropriate therapies were triggered by fast sinus rhythm, atrial fibrillation, and T-wave oversensing. No predictors of inappropriate shocks were identified. Risk factors for appropriate ICD therapy were: (1) recurrent syncope despite b-blocker treatment before ICD implantation, (2) pacemaker therapy before ICD implantation, (3) single-chamber ICD, and (4) noncompliance to b-blockers. In 38 (57%) patients, at least one complication occurred.

Conclusions: ICD therapy is effective in nearly half the patient population; however, the rates of early and late complica­tions are high. Although the number of unnecessary ICD shocks and reimplantation procedures may be lowered by modern programming and increased longevity of newer ICD generators, other adverse events are less likely to be reduced.

Abstract

Background: Implantable cardioverter-defibrillator (ICD) therapy has been proven effective in the prevention of sudden cardiac death, but data on outcomes of ICD therapy in the young and otherwise healthy patients with long QT syndrome (LQTS) are limited.

Aim: We sought to collect data on appropriate and inappropriate ICD discharges, risk factors, and ICD-related complications.

Methods: All LQTS patients implanted with an ICD in 14 centres were investigated. Demographic, clinical, and ICD therapy data were collected.

Results: The study included 67 patients (88% female). Median age at ICD implantation was 31 years (12–77 years). ICD indication was based on resuscitated cardiac arrest in 46 patients, syncope in 18 patients, and malignant family history in three patients. During a median follow-up of 48 months, 39 (58%) patients received one or more ICD therapies. Time to first appropriate discharge was up to 55 months. Inappropriate therapies were triggered by fast sinus rhythm, atrial fibrillation, and T-wave oversensing. No predictors of inappropriate shocks were identified. Risk factors for appropriate ICD therapy were: (1) recurrent syncope despite b-blocker treatment before ICD implantation, (2) pacemaker therapy before ICD implantation, (3) single-chamber ICD, and (4) noncompliance to b-blockers. In 38 (57%) patients, at least one complication occurred.

Conclusions: ICD therapy is effective in nearly half the patient population; however, the rates of early and late complica­tions are high. Although the number of unnecessary ICD shocks and reimplantation procedures may be lowered by modern programming and increased longevity of newer ICD generators, other adverse events are less likely to be reduced.

Get Citation

Keywords

device recalls, ICD outcome, implantable cardioverter-defibrillator, lead dysfunction, long QT syndrome, risk factors

About this article
Title

Implantable cardioverter-defibrillators in patients with long QT syndrome: a multicentre study

Journal

Kardiologia Polska (Polish Heart Journal)

Issue

Vol 76, No 12 (2018)

Pages

1687-1696

Published online

2018-08-17

DOI

10.5603/KP.a2018.0177

Pubmed

30251242

Bibliographic record

Kardiol Pol 2018;76(12):1687-1696.

Keywords

device recalls
ICD outcome
implantable cardioverter-defibrillator
lead dysfunction
long QT syndrome
risk factors

Authors

Agnieszka Zienciuk-Krajka
Maciej Sterliński
Artur Filipecki
Radosław Owczuk
Jacek Bednarek
Maciej Kempa
Sławomir Sielski
Marcin Dziduszko
Przemysław Mitkowski
Jarosław Kaźmierczak
Jacek Kuśnierz
Dariusz Michałkiewicz
Sebastian M. Stec
Marcin Gułaj
Ryszard Majcherek
Andrzej Lubiński
Grzegorz Raczak

References (26)
  1. Priori S, Blomström-Lundqvist C, Mazzanti A, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2015; 36(41): 2793–2867.
  2. Kaufman ES. Arrhythmic risk in congenital long QT syndrome. J Electrocardiol. 2011; 44(6): 645–649.
  3. Schwartz PJ, Moss AJ, Vincent GM, et al. Diagnostic criteria for the long QT syndrome. An update. Circulation. 1993; 88(2): 782–784.
  4. Zareba W, Moss A, Daubert J, et al. Implantable cardioverter defibrillator in high-risk long QT syndrome patients. J Cardiovasc Electrophysiol. 2003; 14(4): 337–341.
  5. Mönnig G, Köbe J, Löher A, et al. Implantable cardioverter-defibrillator therapy in patients with congenital long-QT syndrome: a long-term follow-up. Heart Rhythm. 2005; 2(5): 497–504.
  6. Horner JM, Kinoshita M, Webster TL, et al. Implantable cardioverter defibrillator therapy for congenital long QT syndrome: a single-center experience. Heart Rhythm. 2010; 7(11): 1616–1622.
  7. Schwartz PJ, Spazzolini C, Priori SG, 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): 1272–1282.
  8. Kaufman ES. Saving lives in congenital long QT syndrome: who benefits from implantable cardioverter defibrillator therapy? J Cardiovasc Electrophysiol. 2003; 14(4): 342–343.
  9. Epstein AE, Dimarco JP, Ellenbogen KA, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. Circulation. 2008; 117(21): e350–e408.
  10. Kaufman ES, McNitt S, Moss AJ, et al. Risk of death in the long QT syndrome when a sibling has died. Heart Rhythm. 2008; 5(6): 831–836.
  11. Wichter T, Paul M, Wollmann C, et al. Implantable cardioverter/defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy: single-center experience of long-term follow-up and complications in 60 patients. Circulation. 2004; 109(12): 1503–1508.
  12. Priori SG, Napolitano C, Schwartz PJ, et al. Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers. JAMA. 2004; 292(11): 1341–1344.
  13. Kimbrough J, Moss AJ, Zareba W, et al. Clinical implications for affected parents and siblings of probands with long-QT syndrome. Circulation. 2001; 104(5): 557–562.
  14. Napolitano C, Priori SG, Schwartz PJ, et al. Risk stratification in the long-QT syndrome. N Engl J Med. 2003; 348(19): 1866–1874.
  15. Olde Nordkamp LRA, Wilde AAM, Tijssen JGP, et al. 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(1): 91–100.
  16. Sacher F, Probst V, Maury P, et al. Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study-part 2. Circulation. 2013; 128(16): 1739–1747.
  17. Johnson JN, Tester DJ, Perry J, et al. Prevalence of early-onset atrial fibrillation in congenital long QT syndrome. Heart Rhythm. 2008; 5(5): 704–709.
  18. Wathen MS, DeGroot PJ, Sweeney MO, et al. Prospective randomized multicenter trial of empirical antitachycardia pacing versus shocks for spontaneous rapid ventricular tachycardia in patients with implantable cardioverter-defibrillators: Pacing Fast Ventricular Tachycardia Reduces Shock Therapies (PainFREE Rx II) trial results. Circulation. 2004; 110(17): 2591–2596.
  19. Rudic B, Tülümen E, Borggrefe M, et al. Proarrhythmic effect of “reverse mode switch” in a patient with long-QT syndrome. J Cardiovasc Electrophysiol. 2014; 25(10): 1133–1134.
  20. Di Cori A, Zucchelli G, Romano S, et al. Proarrhythmic effect of pacing mode reprogramation in a patient with a congenital long-QT syndrome. J Cardiovasc Electrophysiol. 2013; 24(11): 1304–1305.
  21. Schellevis MM, VAN Halm VP. Ventricular fibrillation due to automated atrial threshold testing in a patient with an implantable cardioverter defibrillator. Pacing Clin Electrophysiol. 2015; 38(11): 1355–1358.
  22. Schwartz P, Priori S, Spazzolini C, et al. Genotype-Phenotype correlation in the long-qt syndrome. Circulation. 2001; 103(1): 89–95.
  23. Olde Nordkamp LRA, 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): 443–454.
  24. Gaba P, Bos J, Cannon B, et al. Implantable cardioverter-defibrillator explantation for overdiagnosed or overtreated congenital long QT syndrome. Heart Rhythm. 2016; 13(4): 879–885.
  25. Botto GL, Forleo GB, Capucci A, et al. ‘AIAC S-ICD Why Not’ Survey Investigators. The Italian subcutaneous implantable cardioverter-defibrillator survey: S-ICD, why not? Europace. 2017; 19(11): 1826–1832.
  26. Ptaszyński P, Grabowski M, Kowalski O, et al. [Subcutaneous implantable cardioverter-defibrillator in prevention of sudden cardiac death in Poland - opinion paper endorsed by the Polish Cardiac Society Working Group on Heart Rhythm]. Kardiol Pol. 2017; 75(10): 1057–1060.

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

By "Via Medica sp. z o.o." sp.k., Świętokrzyska 73 street, 80–180 Gdańsk, Poland

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail: viamedica@viamedica.pl