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Atrial flutter or fibrillation is the most frequent and life-threatening arrhythmia in myotonic dystrophy.
- Source :
-
Pacing and clinical electrophysiology : PACE [Pacing Clin Electrophysiol] 2014 Mar; Vol. 37 (3), pp. 329-35. Date of Electronic Publication: 2013 Oct 09. - Publication Year :
- 2014
-
Abstract
- Background: Several arrhythmias were reported in myotonic dystrophy (MD).<br />Objectives: To evaluate the prevalence of atrial fibrillation (AF) and atrial flutter (AFL) in MD and the clinical consequences.<br />Methods: One hundred sixty-one patients, mean age 41 ± 14 years, were referred for a type 1 MD. All patients were asymptomatic except four patients and followed during 5 ± 4 years. Electrocardiogram (ECG), echocardiography assessing left ventricular ejection fraction, and Holter monitoring were obtained and repeated.<br />Results: Twenty-seven patients (17%) presented sustained (>1 hour) AF (n = 15) or AFL (n = 12); two of them presented syncope-related 1/1 AFL. In one of them, 16 years of age, cardiac defibrillator was implanted for a diagnosis of ventricular tachycardia, but the true diagnosis was established after inappropriate shocks. AFL ablation was performed in five patients, but four developed AF. The other seven patients with AFL developed AF. During the follow-up, 22 patients died (14%) from cardiac and respiratory failure; eight patients with AF/AFL died (30%) while only 14 without AF/AFL died (10%; P < 0.01). Univariate analysis indicated that age >40 years (death: 48 ± 14 vs 40 ± 8 in alive patients), abnormal ECG, and occurrence of AF/AFL were significant factors of death. At multivariate analysis, AF at ECG (odds ratio: 3.12) and age >40 (odds ratio: 3.14) were the sole independent variables predicting death.<br />Conclusions: AF and AFL were frequent in MD and increased mortality. AFL could present as 1/1 AFL with a poor tolerance and a risk of misdiagnosis despite frequent conduction disturbances. This arrhythmia could explain wide QRS tachycardia occurring in MD and interpreted as VT.<br /> (©2013, The Authors. Journal compilation ©2013 Wiley Periodicals, Inc.)
- Subjects :
- Adolescent
Adult
Age Distribution
Aged
Atrial Fibrillation therapy
Atrial Flutter therapy
Comorbidity
Electrocardiography statistics & numerical data
Female
France
Humans
Male
Middle Aged
Myotonic Dystrophy therapy
Proportional Hazards Models
Reproducibility of Results
Risk Assessment
Sensitivity and Specificity
Sex Distribution
Survival Rate
Young Adult
Atrial Fibrillation diagnosis
Atrial Fibrillation mortality
Atrial Flutter diagnosis
Atrial Flutter metabolism
Myotonic Dystrophy diagnosis
Myotonic Dystrophy mortality
Subjects
Details
- Language :
- English
- ISSN :
- 1540-8159
- Volume :
- 37
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Pacing and clinical electrophysiology : PACE
- Publication Type :
- Academic Journal
- Accession number :
- 24117873
- Full Text :
- https://doi.org/10.1111/pace.12260