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Girish V Nair - One of the best experts on this subject based on the ideXlab platform.
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electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1
The New England Journal of Medicine, 2008Co-Authors: William J Groh, Miriam R Groh, Chandan Saha, John C Kincaid, Zachary Simmons, Emma Ciafaloni, Rahman Pourmand, Richard F Otten, Deepak Bhakta, Girish V NairAbstract:Background Sudden death can occur as a consequence of cardiac-conduction abnormalities in the neuromuscular disease myotonic dystrophy type 1. The determinants of the risk of sudden death remain imprecise. Methods We assessed whether the electrocardiogram (ECG) was useful in predicting sudden death in 406 adult patients with genetically confirmed myotonic dystrophy type 1. A patient was characterized as having a severe abnormality if the ECG had at least one of the following features: rhythm other than sinus, PR interval of 240 msec or more, QRS duration of 120 msec or more, or second-degree or Third-Degree Atrioventricular Block. Results Patients with severe abnormalities according to the entry ECG were older than patients without severe abnormalities, had more severe skeletal-muscle impairment, and were more likely to have heart failure, left ventricular systolic dysfunction, or atrial tachyarrhythmia. Such patients were more likely to receive a pacemaker or an implantable cardioverter–defibrillator dur...
M William P H J Groh - One of the best experts on this subject based on the ideXlab platform.
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pacemaker and implantable cardioverter defibrillator use in a us myotonic dystrophy type 1 population
Journal of Cardiovascular Electrophysiology, 2011Co-Authors: M Deepak D Bhakta, Changyu Shen, M Jack D Kron, E Andrew M D Epstein, M Robert M D Pascuzzi, M William P H J GrohAbstract:Pacemakers and ICDs in Myotonic Dystrophy. Introduction: We assessed implant rates, indications, characteristics, and outcomes in patients with the neuromuscular disease, myotonic dystrophy type 1 (DM1) receiving a pacemaker or an implantable cardioverter-defibrillator (ICD). Methods and Results: Device use was evaluated in a prospective, multicenter registry of 406 genetically confirmed adult patients followed for 9.5 ± 3.2 years. Forty-six (11.3%) had or received a pacemaker and 21 (5.2%) received an ICD. Devices were primarily implanted for asymptomatic conduction abnormalities and left ventricular (LV) systolic dysfunction. However, 7 (15.2%) pacemakers were implanted for Third-Degree Atrioventricular Block and 6 (28.6%) ICDs were implanted for ventricular tachyarrhythmias (ventricular tachycardia [VT] or fibrillation [VF]). Patients receiving devices were older and more frequently had heart failure, LV systolic dysfunction, atrial tachyarrhythmias, and ECG conduction abnormalities compared to nondevice patients. Five (10.9%) pacemaker patients underwent upgrade to an ICD, 3 for LV systolic dysfunction, 1 for VT/VF, and 1 for progressive conduction disease. Seventeen (27.4%) of the 62 patients with devices were pacemaker-dependent at last follow-up. Three (14.3%) ICD patients had appropriate therapies. Twenty-four (52.2%) pacemaker patients died including 13 of respiratory failure and 7 of sudden death. Seven (33.3%) ICD patients died including 2 of respiratory failure and 3 of sudden death. The patients with ICDs and sudden death all had LV systolic dysfunction and 1 death was documented due to inappropriate therapies. Conclusions: DM1 patients commonly receive antiarrhythmia devices. The risk of VT/VF and sudden death suggests that ICDs rather than pacemakers should be considered for these patients. (J Cardiovasc Electrophysiol, Vol. 22, pp. 1369-1375, December 2011)
Bernardelli Emanuela - One of the best experts on this subject based on the ideXlab platform.
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Registro Italiano Pacemaker e Defibrillatori: Bollettino Periodico 2016 Associazione Italiana di Aritmologia e Cardiostimolazione
Il Pensiero Scientifico Editore s.r.l., 2018Co-Authors: Proclemer Alessandro, Zecchin Massimo, Boriani Giuseppe, Facchin Domenico, Rebellato Luca, Ghidina Marco, Bianco Giulia, Bernardelli Emanuela, Pucher ElsaAbstract:Background: The pacemaker (PM) and implantable cardioverter-defibrillator (ICD) registry of the Italian Association of Arrhythmology and Cardiac Pacing (AIAC) monitors the main epidemiological data in real-world practice. The survey for the 2016 activity collects information about demographics, clinical characteristics, main indications for PM/ICD therapy and device types from the Italian collaborating centers. Methods: The registry collects prospectively national PM and ICD implantation activity on the basis of European cards. Results: PM Registry: data about 23 496 PM implantations were collected (19 003 first implant and 4493 replacements). The number of collaborating centers was 204. Median age of treated patients was 81 years (75 quartile I; 86 quartile III). ECG indications included Atrioventricular conduction disorders in 39.8% of first PM implants, sick sinus syndrome in 22.1%, atrial fibrillation plus bradycardia in 14.1%, other in 24.0%. Among Atrioventricular conduction defects, Third-Degree Atrioventricular Block was the most common type (22.18% of first implants). Use of single-chamber PMs was reported in 26.9% of first implants, of dual-chamber PMs in 65.0%, of PMs with cardiac resynchronization therapy (CRT) in 1.5%, and of single lead atrial-synchronized ventricular stimulation (VDD/R PMs) in 6.6%. ICD Registry: data about 20 350 ICD implantations were collected (14 763 first implants and 5587 replacements). The number of collaborating centers was 430. Median age of treated patients was 72 years (63 quartile I; 78 quartile III]. Primary prevention indication was reported in 79.0% of first implants, secondary prevention in 21.0% (cardiac arrest in 7.9%). A single-chamber ICD was used in 32.2% of first implants, dual-chamber in 31.1% and biventricular in 36.7%. Conclusions: The PM and ICD Registry appears fundamental for monitoring PM and ICD utilization on a large national scale with rigorous examination of demographics and clinical indications. The PM Registry showed stable electrocardiographic and symptom indications, with an important prevalence of dual-chamber pacing. The use of CRT-PM regards a very limited number of patients. The ICD Registry documented a large use of prophylactic and biventricular ICD, reflecting a favorable adherence to trials and guidelines in clinical practice. \ua9 2018 Il Pensiero Scientifico Editore
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Registro Italiano Pacemaker e Defibrillatori: Bollettino Periodico 2016 Associazione Italiana di Aritmologia e Cardiostimolazione
2018Co-Authors: Proclemer Alessandro, Zecchin Massimo, Boriani Giuseppe, Facchin Domenico, Rebellato Luca, Ghidina Marco, Bianco Giulia, Bernardelli Emanuela, Pucher ElsaAbstract:Background: The pacemaker (PM) and implantable cardioverter-defibrillator (ICD) registry of the Italian Association of Arrhythmology and Cardiac Pacing (AIAC) monitors the main epidemiological data in real-world practice. The survey for the 2016 activity collects information about demographics, clinical characteristics, main indications for PM/ICD therapy and device types from the Italian collaborating centers. Methods: The registry collects prospectively national PM and ICD implantation activity on the basis of European cards. Results: PM Registry: data about 23 496 PM implantations were collected (19 003 first implant and 4493 replacements). The number of collaborating centers was 204. Median age of treated patients was 81 years (75 quartile I; 86 quartile III). ECG indications included Atrioventricular conduction disorders in 39.8% of first PM implants, sick sinus syndrome in 22.1%, atrial fibrillation plus bradycardia in 14.1%, other in 24.0%. Among Atrioventricular conduction defects, Third-Degree Atrioventricular Block was the most common type (22.18% of first implants). Use of single-chamber PMs was reported in 26.9% of first implants, of dual-chamber PMs in 65.0%, of PMs with cardiac resynchronization therapy (CRT) in 1.5%, and of single lead atrial-synchronized ventricular stimulation (VDD/R PMs) in 6.6%. ICD Registry: data about 20 350 ICD implantations were collected (14 763 first implants and 5587 replacements). The number of collaborating centers was 430. Median age of treated patients was 72 years (63 quartile I; 78 quartile III]. Primary prevention indication was reported in 79.0% of first implants, secondary prevention in 21.0% (cardiac arrest in 7.9%). A single-chamber ICD was used in 32.2% of first implants, dual-chamber in 31.1% and biventricular in 36.7%. Conclusions: The PM and ICD Registry appears fundamental for monitoring PM and ICD utilization on a large national scale with rigorous examination of demographics and clinical indications. The PM Registry showed stable electrocardiographic and symptom indications, with an important prevalence of dual-chamber pacing. The use of CRT-PM regards a very limited number of patients. The ICD Registry documented a large use of prophylactic and biventricular ICD, reflecting a favorable adherence to trials and guidelines in clinical practice
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Registro Italiano pacemaker e defibrillatori bollettino periodico 2015: Associazione Italiana di Aritmologia e Cardiostimolazione
Il Pensiero Scientifico Editore s.r.l., 2017Co-Authors: Proclemer Alessandro, Zecchin Massimo, Boriani Giuseppe, Facchin Domenico, Rebellato Luca, Ghidina Marco, Bianco Giulia, Botto, Giovanni Luca, Bernardelli EmanuelaAbstract:Background: The pacemaker (PM) and implantable cardioverter-defibrillator (ICD) Registry of the Italian Association of Arrhythmology and Cardiac Pacing (AIAC) monitors the main epidemiological data in real-world practice. The survey for the 2015 activity collects information about demographics, clinical characteristics, main indications for PM/ICD therapy and device types from the Italian collaborating centers. Methods: The Registry collects prospectively national PM and ICD implantation activity on the basis of European cards. Results: PM Registry: data about 24 285 PM implantations were collected (19 194 first implant and 5091 replacements). The number of collaborating centers was 218. Median age of treated patients was 81 years (75 quartile I; 86 quartile III). ECG indications included Atrioventricular conduction disorders in 42.8% of first PM implants, sick sinus syndrome in 22.9%, atrial fibrillation plus bradycardia in 15.1%, other in 19.2%. Among Atrioventricular conduction defects, Third-Degree Atrioventricular Block was the most common type (23.8% of first implants). Use of single-chamber PMs was reported in 26.9% of first implants, of dual-chamber PMs in 63.4%, of PMs with cardiac resynchronization therapy (CRT) in 1.8%, and of single lead atrial-synchronized ventricular stimulation (VDD/R PMs) in 7.9%. ICD Registry: data about 15 363 ICD implantations were collected (11 453 first implants and 3910 replacements). The number of collaborating centers was 434. Median age of treated patients was 71 years (63 quartile I; 78 quartile III]. Primary prevention indication was reported in 77.3% of first implants, secondary prevention in 22.7% (cardiac arrest in 8.0%). A single-chamber ICD was used in 29.3% of first implants, dual-chamber in 34.6% and biventricular in 36.1%. Conclusions: The PM and ICD Registry appears fundamental for monitoring PM and ICD utilization on a large national scale with rigorous examination of demographics and clinical indications. The PM Registry showed stable electrocardiographic and symptom indications, with an important prevalence of dual-chamber pacing. The use of CRT-PM regards a very limited number of patients. The ICD Registry documented a large use of prophylactic and biventricular ICD, reflecting a favorable adherence to trials and guidelines in clinical practice. \ua9 2017 Il Pensiero Scientifico Editore
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Registro Italiano pacemaker e defibrillatori bollettino periodico 2015: Associazione Italiana di Aritmologia e Cardiostimolazione
2017Co-Authors: Proclemer Alessandro, Zecchin Massimo, Boriani Giuseppe, Facchin Domenico, Rebellato Luca, Ghidina Marco, Bianco Giulia, Botto, Giovanni Luca, Bernardelli EmanuelaAbstract:Background: The pacemaker (PM) and implantable cardioverter-defibrillator (ICD) Registry of the Italian Association of Arrhythmology and Cardiac Pacing (AIAC) monitors the main epidemiological data in real-world practice. The survey for the 2015 activity collects information about demographics, clinical characteristics, main indications for PM/ICD therapy and device types from the Italian collaborating centers. Methods: The Registry collects prospectively national PM and ICD implantation activity on the basis of European cards. Results: PM Registry: data about 24 285 PM implantations were collected (19 194 first implant and 5091 replacements). The number of collaborating centers was 218. Median age of treated patients was 81 years (75 quartile I; 86 quartile III). ECG indications included Atrioventricular conduction disorders in 42.8% of first PM implants, sick sinus syndrome in 22.9%, atrial fibrillation plus bradycardia in 15.1%, other in 19.2%. Among Atrioventricular conduction defects, Third-Degree Atrioventricular Block was the most common type (23.8% of first implants). Use of single-chamber PMs was reported in 26.9% of first implants, of dual-chamber PMs in 63.4%, of PMs with cardiac resynchronization therapy (CRT) in 1.8%, and of single lead atrial-synchronized ventricular stimulation (VDD/R PMs) in 7.9%. ICD Registry: data about 15 363 ICD implantations were collected (11 453 first implants and 3910 replacements). The number of collaborating centers was 434. Median age of treated patients was 71 years (63 quartile I; 78 quartile III]. Primary prevention indication was reported in 77.3% of first implants, secondary prevention in 22.7% (cardiac arrest in 8.0%). A single-chamber ICD was used in 29.3% of first implants, dual-chamber in 34.6% and biventricular in 36.1%. Conclusions: The PM and ICD Registry appears fundamental for monitoring PM and ICD utilization on a large national scale with rigorous examination of demographics and clinical indications. The PM Registry showed stable electrocardiographic and symptom indications, with an important prevalence of dual-chamber pacing. The use of CRT-PM regards a very limited number of patients. The ICD Registry documented a large use of prophylactic and biventricular ICD, reflecting a favorable adherence to trials and guidelines in clinical practice
William J Groh - One of the best experts on this subject based on the ideXlab platform.
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electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1
The New England Journal of Medicine, 2008Co-Authors: William J Groh, Miriam R Groh, Chandan Saha, John C Kincaid, Zachary Simmons, Emma Ciafaloni, Rahman Pourmand, Richard F Otten, Deepak Bhakta, Girish V NairAbstract:Background Sudden death can occur as a consequence of cardiac-conduction abnormalities in the neuromuscular disease myotonic dystrophy type 1. The determinants of the risk of sudden death remain imprecise. Methods We assessed whether the electrocardiogram (ECG) was useful in predicting sudden death in 406 adult patients with genetically confirmed myotonic dystrophy type 1. A patient was characterized as having a severe abnormality if the ECG had at least one of the following features: rhythm other than sinus, PR interval of 240 msec or more, QRS duration of 120 msec or more, or second-degree or Third-Degree Atrioventricular Block. Results Patients with severe abnormalities according to the entry ECG were older than patients without severe abnormalities, had more severe skeletal-muscle impairment, and were more likely to have heart failure, left ventricular systolic dysfunction, or atrial tachyarrhythmia. Such patients were more likely to receive a pacemaker or an implantable cardioverter–defibrillator dur...
Gregg W Stone - One of the best experts on this subject based on the ideXlab platform.
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incidence predictors and outcomes of high grade Atrioventricular Block in patients with st segment elevation myocardial infarction undergoing primary percutaneous coronary intervention from the horizons ami trial
American Journal of Cardiology, 2017Co-Authors: Ioanna Kosmidou, Bjorn Redfors, Rushad Dordi, Jose Dizon, Thomas Mcandrew, Roxana Mehran, Ori Benyehuda, Gary S Mintz, Gregg W StoneAbstract:High-grade Atrioventricular Block (HAVB) is historically considered a marker of worse outcomes in patients with ST-segment elevation myocardial infarction (STEMI). However, the predictors and prognostic impact of HAVB in the primary percutaneous coronary intervention (PCI) era remain poorly understood. We sought to describe the characteristics and predictors of HAVB in patients undergoing primary PCI in STEMI and to assess the prognostic significance of HAVB in the contemporary reperfusion era. The present analysis includes 3,115 patients presenting with STEMI from the Harmonizing Outcomes with Revascularization and Stents in Acute Myocardial Infarction trial who underwent primary PCI. Outcomes were examined according to the presence of HAVB on a presenting electrocardiogram, as interpreted by an independent electrocardiography core laboratory. HAVB (second-degree Mobitz II or Third-Degree Atrioventricular Block) was present at baseline in 46 patients (1.5%). Independent predictors of HAVB included increased age, diabetes mellitus, right coronary artery occlusion, sum of ST-segment deviation, and baseline Thrombolysis In Myocardial Infarction flow 0/1. Thrombolysis In Myocardial Infarction flow 3 was restored in 83.7% and 91.5% of patients with versus without baseline HAVB respectively (p = 0.06). Mortality rate was significantly higher in patients with versus without HAVB at 30-day, 1-, and 3-year follow-ups (unadjusted hazard ratio [HR] 3.83, 95% CI 1.40 to 10.48; unadjusted HR 4.37, 95% CI 2.09 to 9.38 and unadjusted HR 2.78, 95% CI 1.31 to 5.91, respectively). After covariate adjustment, mortality rate was significantly higher in patients with HAVB at 1 year (adjusted HR 2.45, 95% CI 1.09 to 5.50, p = 0.03) but not at 30 days (adjusted HR 1.70, 95% CI 0.58 to 5.01, p = 0.33) or 3 years (adjusted HR 0.71 to 3.41, p = 0.27). In conclusion, HAVB is a rare complication of STEMI but remains associated with increased mortality, even after primary PCI.