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

التفاصيل البيبلوغرافية
العنوان: 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
المؤلفون: Pedro Brugada, Majid Al-Fayyadh, Carla Spazzolini, Lia Crotti, Arthur A.M. Wilde, Gerold Mönnig, Peter J. Schwartz, Luc De Roy, Lauri Toivonen, Paul A. Brink, Maurizio Landolina, Maurizio Gasparini, Reinoud E. Knops, Alessandro Vicentini, Stefan H. Hohnloser, Luc Jordaens, Christina Holmgren, Isabelle Denjoy, Silvia G. Priori, Martin Borggrefe
المساهمون: Schwartz, P, Spazzolini, C, Priori, S, Crotti, L, Vicentini, A, Landolina, M, Gasparini, M, Wilde, A, Knops, R, Denjoy, I, Toivonen, L, Mönnig, G, Al-Fayyadh, M, Jordaens, L, Borggrefe, M, Holmgren, C, Brugada, P, De Roy, L, Hohnloser, S, Brink, P, ACS - Amsterdam Cardiovascular Sciences, Cardiology, CCA -Cancer Center Amsterdam, APH - Amsterdam Public Health, Internal Medicine Specializations, Heartrhythmmanagement, Cardio-vascular diseases, USL-B - Réseau Interdisciplinarité et Société (RIS), UCL - SSS/IREC/MONT - Pôle Mont Godinne, UCL - (MGD) Service de cardiologie
المصدر: Circulation, 122(13), 1272-1282. Lippincott Williams and Wilkins
Circulation, 122(13), 1272-1282. Lippincott Williams & Wilkins
Circulation, Vol. 122, no. 13, p. 1272-1782 (2010)
بيانات النشر: LIPPINCOTT WILLIAMS & WILKINS, 2010.
سنة النشر: 2010
مصطلحات موضوعية: Adult, Male, implantable cardioverter-defibrillator, long qt syndrome, European Long-QT Syndrome Implantable Cardioverter-Defibrillator (LQTS ICD) Registry, medicine.medical_specialty, Heart disease, Adolescent, Genotype, Defibrillation, Heart block, medicine.medical_treatment, Long QT syndrome, 030204 cardiovascular system & hematology, arrhythmia, Asymptomatic, QT interval, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Physiology (medical), Internal medicine, medicine, Humans, genetics, 030212 general & internal medicine, Registries, Adverse effect, Child, business.industry, Defibrillators, Implantable/adverse effects, cild, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, Long QT Syndrome/genetics, Long QT Syndrome/therapy, Middle Aged, medicine.disease, Implantable cardioverter-defibrillator, defibrillation, 3. Good health, Defibrillators, Implantable, Europe, Long QT Syndrome, Treatment Outcome, Cardiology, young adult, Female, medicine.symptom, Cardiology and Cardiovascular Medicine, business, heart arrest
الوصف: 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 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.
وصف الملف: STAMPA
اللغة: English
تدمد: 0009-7322
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::af4af9514696ee55f285a702c6dc540b
http://hdl.handle.net/10281/189635
حقوق: OPEN
رقم الأكسشن: edsair.doi.dedup.....af4af9514696ee55f285a702c6dc540b
قاعدة البيانات: OpenAIRE