1. Pacemaker and Implantable Cardioverter-Defibrillator Use in a US Myotonic Dystrophy Type 1 Population.
- Author
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BHAKTA, DEEPAK, SHEN, CHANGYU, KRON, JACK, EPSTEIN, ANDREW E., PASCUZZI, ROBERT M., and GROH, WILLIAM J.
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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 - 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]
- Published
- 2011
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