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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

(2010) CIRCULATION. 122(13). p.1272-1282
Author
Organization
Abstract
Background: A rapidly growing number of long-QT syndrome (LQTS) patients are being treated with an implantable cardioverter-defibrillator (ICD). ICDs may pose problems, especially in the young. We sought to determine the characteristics of the LQTS patients receiving an ICD, the indications, and the aftermath. Methods and Results: The study population included 233 patients. Beginning in 2002, data were collected prospectively. Female patients (77%) and LQT3 patients (22% of genotype positive) were overrepresented; mean QTc was 516 +/- 65 milliseconds; mean age at implantation was 30 +/- 17 years; and genotype was known in 59% of patients. Unexpectedly, 9% of patients were asymptomatic before implantation. Asymptomatic patients, almost absent among LQT1 and LQT2 patients, represented 45% of LQT3 patients. Patients with cardiac symptoms made up 91% of all study participants, but only 44% had cardiac arrest before ICD implantation. In addition, 41% of patients received an ICD without having first been on LQTS therapy. During follow-up, 4.6 +/- 3.2 years, at least 1 appropriate shock was received by 28% of patients, and adverse events occurred in 25%. Appropriate ICD therapies were predicted by age <20 years at implantation, a QTc >500 milliseconds, prior cardiac arrest, and cardiac events despite therapy; within 7 years, appropriate shocks occurred in no patients with none of these factors and in 70% of those with all factors. Conclusions: Reflecting previous concepts, ICDs were implanted in some LQTS patients whose high risk now appears questionable. Refined criteria for implantation, reassessment of pros and cons, ICD reprogramming, and consideration for other existing therapeutic options are necessary.
Keywords
defibrillation, genetics, arrhythmia, heart arrest, long QT syndrome, CHILDREN, Q-T SYNDROME

Citation

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MLA
Schwartz, Peter J 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 122.13 (2010): 1272–1282. Print.
APA
Schwartz, P. J., Spazzolini, C., Priori, S. G., Crotti, L., Vicentini, A., Landolina, M., Gasparini, M., et al. (2010). 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, 122(13), 1272–1282.
Chicago author-date
Schwartz, Peter J, Carla Spazzolini, Silvia G Priori, Lia Crotti, Alessandro Vicentini, Maurizio Landolina, Maurizio Gasparini, et al. 2010. “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 122 (13): 1272–1282.
Chicago author-date (all authors)
Schwartz, Peter J, Carla Spazzolini, Silvia G Priori, Lia Crotti, Alessandro Vicentini, Maurizio Landolina, Maurizio Gasparini, Arthur AM Wilde, Reinoud E Knops, Isabelle Denjoy, Lauri Toivonen, Gerold Mönnig, Majid Al-Fayyadh, Luc Jordaens, Martin Borggrefe, Christina Holmgren, Pedro Brugada, Luc De Roy, Stefan H Hohnloser, and Paul A Brink. 2010. “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 122 (13): 1272–1282.
Vancouver
1.
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.
IEEE
[1]
P. J. Schwartz 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, vol. 122, no. 13, pp. 1272–1282, 2010.
@article{8094114,
  abstract     = {Background: A rapidly growing number of long-QT syndrome (LQTS) patients are being treated with an implantable cardioverter-defibrillator (ICD). ICDs may pose problems, especially in the young. We sought to determine the characteristics of the LQTS patients receiving an ICD, the indications, and the aftermath. 
Methods and Results: The study population included 233 patients. Beginning in 2002, data were collected prospectively. Female patients (77%) and LQT3 patients (22% of genotype positive) were overrepresented; mean QTc was 516 +/- 65 milliseconds; mean age at implantation was 30 +/- 17 years; and genotype was known in 59% of patients. Unexpectedly, 9% of patients were asymptomatic before implantation. Asymptomatic patients, almost absent among LQT1 and LQT2 patients, represented 45% of LQT3 patients. Patients with cardiac symptoms made up 91% of all study participants, but only 44% had cardiac arrest before ICD implantation. In addition, 41% of patients received an ICD without having first been on LQTS therapy. During follow-up, 4.6 +/- 3.2 years, at least 1 appropriate shock was received by 28% of patients, and adverse events occurred in 25%. Appropriate ICD therapies were predicted by age <20 years at implantation, a QTc >500 milliseconds, prior cardiac arrest, and cardiac events despite therapy; within 7 years, appropriate shocks occurred in no patients with none of these factors and in 70% of those with all factors. 
Conclusions: Reflecting previous concepts, ICDs were implanted in some LQTS patients whose high risk now appears questionable. Refined criteria for implantation, reassessment of pros and cons, ICD reprogramming, and consideration for other existing therapeutic options are necessary.},
  author       = {Schwartz, Peter J and Spazzolini, Carla and Priori, Silvia G and Crotti, Lia and Vicentini, Alessandro and Landolina, Maurizio and Gasparini, Maurizio and Wilde, Arthur AM and Knops, Reinoud E and Denjoy, Isabelle and Toivonen, Lauri and Mönnig, Gerold and Al-Fayyadh, Majid and Jordaens, Luc and Borggrefe, Martin and Holmgren, Christina and Brugada, Pedro and De Roy, Luc and Hohnloser, Stefan H and Brink, Paul A},
  issn         = {0009-7322},
  journal      = {CIRCULATION},
  keywords     = {defibrillation,genetics,arrhythmia,heart arrest,long QT syndrome,CHILDREN,Q-T SYNDROME},
  language     = {eng},
  number       = {13},
  pages        = {1272--1282},
  title        = {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},
  url          = {http://dx.doi.org/10.1161/CIRCULATIONAHA.110.950147},
  volume       = {122},
  year         = {2010},
}

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