Pacemaker and implantable cardioverter-defibrillator use in a US myotonic dystrophy type 1 population

Deepak Bhakta, Changyu Shen, Jack Kron, Andrew E. Epstein, Robert Pascuzzi, William Groh

Research output: Contribution to journalArticle

39 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)1369-1375
Number of pages7
JournalJournal of Cardiovascular Electrophysiology
Volume22
Issue number12
DOIs
StatePublished - Dec 2011

Fingerprint

Myotonic Dystrophy
Implantable Defibrillators
Population
Left Ventricular Dysfunction
Sudden Death
Ventricular Fibrillation
Equipment and Supplies
Ventricular Tachycardia
Tachycardia
Respiratory Insufficiency
Neuromuscular Diseases
Atrioventricular Block
Registries
Electrocardiography
Heart Failure

Keywords

  • arrhythmia
  • cardiomyopathy
  • genetics
  • ICDs
  • myotonic dystrophy
  • pacemaker
  • sudden death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Pacemaker and implantable cardioverter-defibrillator use in a US myotonic dystrophy type 1 population. / Bhakta, Deepak; Shen, Changyu; Kron, Jack; Epstein, Andrew E.; Pascuzzi, Robert; Groh, William.

In: Journal of Cardiovascular Electrophysiology, Vol. 22, No. 12, 12.2011, p. 1369-1375.

Research output: Contribution to journalArticle

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