Who Are the Long-QT Syndrome Patients Who Receive an Implantable Cardioverter-Defibrillator and What Happens to Them?
-
- Peter J. Schwartz
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Carla Spazzolini
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Silvia G. Priori
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Lia Crotti
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Alessandro Vicentini
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Maurizio Landolina
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Maurizio Gasparini
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Arthur A.M. Wilde
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Reinoud E. Knops
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Isabelle Denjoy
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Lauri Toivonen
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Gerold Mönnig
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Majid Al-Fayyadh
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Luc Jordaens
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Martin Borggrefe
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Christina Holmgren
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Pedro Brugada
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Luc De Roy
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Stefan H. Hohnloser
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
-
- Paul A. Brink
- From the Section of Cardiology, Department of Lung, Blood, and Heart, University of Pavia, Pavia, Italy (P.J.S., S.G.P., L.C.) Department of Cardiology (P.J.S., C.S., L.C., A.V., M.L.) and Molecular Cardiology Laboratory (P.J.S., L.C.), IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy; Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy (P.J.S.); Department of Internal Medicine (P.J.S., P.A.B.), University of Stellenbosch, Stellenbosch, South Africa;...
書誌事項
- タイトル別名
-
- Data From the European Long-QT Syndrome Implantable Cardioverter-Defibrillator (LQTS ICD) Registry
抄録
<jats:sec> <jats:title>Background—</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Methods and Results—</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions—</jats:title> <jats:p>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.</jats:p> </jats:sec>
収録刊行物
-
- Circulation
-
Circulation 122 (13), 1272-1282, 2010-09-28
Ovid Technologies (Wolters Kluwer Health)