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Pacemaker and Implantable Cardioverter‐Defibrillator Use in a US Myotonic Dystrophy Type 1 Population
Author(s) -
BHAKTA DEEPAK,
SHEN CHANGYU,
KRON JACK,
EPSTEIN ANDREW E.,
PASCUZZI ROBERT M.,
GROH WILLIAM J.
Publication year - 2011
Publication title -
journal of cardiovascular electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.193
H-Index - 138
eISSN - 1540-8167
pISSN - 1045-3873
DOI - 10.1111/j.1540-8167.2011.02200.x
Subject(s) - medicine , cardiology , myotonic dystrophy , implantable cardioverter defibrillator , sudden cardiac death , ventricular fibrillation , ventricular tachycardia , sudden death , heart failure , atrial fibrillation , asymptomatic
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)