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Pacemaker and Implantable Cardioverter-Defibrillator Use in a US Myotonic Dystrophy Type 1 Population.
- Source :
-
Journal of Cardiovascular Electrophysiology . Dec2011, Vol. 22 Issue 12, p1369-1375. 7p. 2 Charts, 2 Graphs. - Publication Year :
- 2011
-
Abstract
- 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) [ABSTRACT FROM AUTHOR]
- Subjects :
- *ARRHYTHMIA
*CARDIAC pacemakers
*CHI-squared test
*CONFIDENCE intervals
*ELECTROCARDIOGRAPHY
*FISHER exact test
*IMPLANTABLE cardioverter-defibrillators
*LONGITUDINAL method
*MEDICAL cooperation
*MYOTONIA atrophica
*SCIENTIFIC observation
*HEALTH outcome assessment
*RESEARCH
*RESEARCH funding
*SURVIVAL analysis (Biometry)
*T-test (Statistics)
*RELATIVE medical risk
*TREATMENT effectiveness
*PROPORTIONAL hazards models
*DATA analysis software
*DESCRIPTIVE statistics
CARDIAC arrest prevention
Subjects
Details
- Language :
- English
- ISSN :
- 10453873
- Volume :
- 22
- Issue :
- 12
- Database :
- Academic Search Index
- Journal :
- Journal of Cardiovascular Electrophysiology
- Publication Type :
- Academic Journal
- Accession number :
- 69627115
- Full Text :
- https://doi.org/10.1111/j.1540-8167.2011.02200.x