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Atrio-ventricular block requiring pacemaker in patients with late onset Pompe disease.
- Source :
-
Neuromuscular disorders : NMD [Neuromuscul Disord] 2014 Jul; Vol. 24 (7), pp. 648-50. Date of Electronic Publication: 2014 Apr 24. - Publication Year :
- 2014
-
Abstract
- Enzyme replacement therapy consistently improves cardiac function in infantile and juvenile onset patients with Pompe disease and cardiomyopathy, but is apparently not effective in preventing rhythm disorders, an emerging cardiac phenotype in long term survivors. In patients with late onset Pompe disease cardiomyopathy is an exceptional finding while heart rhythm disorders seem to be more frequent. We retrospectively identified, among a cohort of 131 French late onset Pompe disease patients, four patients with severe atrio-ventricular blocks requiring pacemaker implantation. These patients had no other risk factors for cardiovascular diseases or cardiomyopathy. In one patient the atrioventricular block was discovered while still asymptomatic. Cardiac conduction defects are relatively rare in late onset Pompe disease and may occur even in absence of cardiac symptoms or EKG abnormalities. However because of the possible life-threatening complications associated with these conduction defects, cardiac follow-up in patients with late onset Pompe disease should include periodical Holter-EKG monitoring.<br /> (Copyright © 2014 Elsevier B.V. All rights reserved.)
- Subjects :
- Adult
Age of Onset
Echocardiography
Fatal Outcome
Female
France
Heart physiopathology
Humans
Male
Middle Aged
Retrospective Studies
Atrioventricular Block physiopathology
Atrioventricular Block therapy
Glycogen Storage Disease Type II physiopathology
Glycogen Storage Disease Type II therapy
Pacemaker, Artificial
Subjects
Details
- Language :
- English
- ISSN :
- 1873-2364
- Volume :
- 24
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Neuromuscular disorders : NMD
- Publication Type :
- Academic Journal
- Accession number :
- 24844452
- Full Text :
- https://doi.org/10.1016/j.nmd.2014.04.005