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Cardiac Abnormalities in Type 1 Facioscapulohumeral Muscular Dystrophy
- Source :
- Journal of Clinical Neuromuscular Disease, Journal of Clinical Neuromuscular Disease, Lippincott, Williams & Wilkins, 2017, 18 (4), pp.199-206. ⟨10.1097/CND.0000000000000144⟩
- Publication Year :
- 2017
- Publisher :
- Ovid Technologies (Wolters Kluwer Health), 2017.
-
Abstract
- International audience; Objectives: We conducted a retrospective study to characterize the cardiac complications in patients with genetically confirmed type 1 facioscapulohumeral dystrophy. Methods: We reviewed baseline cardiac investigations, including electrocardiogram, Holter electrocardiogram and echocardiogram, as well as cardiac complications that occurred during follow-up in 56 adult patients (37 men, mean duration of disease: 20 years). Results: Baseline evaluation revealed minor cardiac anomalies in 23 patients including incomplete right bundle branch block (iRBBB) in 13 patients (23%). Over a mean follow-up period of 7.2 years, there was no cardiac death, no patient developed cardiomyopathy, and 28 patients (50%) experienced cardiac anomalies. Among these patients, 3 had one or more major events (heart failure and/or atrial fibrillation). The remaining 25 patients presented minor cardiac anomalies of which iRBBB was the most frequent (25%). Conclusions: Cardiac anomalies identified during the follow-up of patients with type 1 facioscapulohumeral dystrophy are mainly minor anomalies, dominated by the iRBBB.
- Subjects :
- Adult
Male
medicine.medical_specialty
Heart Diseases
[SDV]Life Sciences [q-bio]
Vital Capacity
Cardiomyopathy
[SHS.PSY]Humanities and Social Sciences/Psychology
030204 cardiovascular system & hematology
Electrocardiography
Young Adult
03 medical and health sciences
0302 clinical medicine
Risk Factors
Forced Expiratory Volume
Internal medicine
medicine
Humans
Facioscapulohumeral muscular dystrophy
Longitudinal Studies
Muscular dystrophy
Aged
Retrospective Studies
Aged, 80 and over
medicine.diagnostic_test
business.industry
[SCCO.NEUR]Cognitive science/Neuroscience
Microfilament Proteins
Nuclear Proteins
RNA-Binding Proteins
Dystrophy
Retrospective cohort study
Atrial fibrillation
General Medicine
Middle Aged
medicine.disease
Muscular Dystrophy, Facioscapulohumeral
3. Good health
Neurology
Heart failure
cardiovascular system
Cardiology
Female
Neurology (clinical)
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 15220443
- Volume :
- 18
- Database :
- OpenAIRE
- Journal :
- Journal of Clinical Neuromuscular Disease
- Accession number :
- edsair.doi.dedup.....d2da358bc00d05c0bbb5e75acb19b37b