Facioscapulohumeral Muscular Dystrophy and Occurrence of Heart Arrhythmia

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Abstract

Background: Subjects with facioscapulohumeral muscular dystrophy (FSHD) do not generally suffer from significant cardiac symptoms. Although with heterogeneous results, studies reported to date indicate that heart alterations unrelated to cardiomyopathy are possible in FSHD. Patients and Methods: We describe the findings of a multicenter investigation aimed at detecting cardiac abnormalities in 83 FSHD patients, 44 males and 39 females with a mean age of 47 years. All patients underwent clinical heart examination, 12-lead electrocardiography and 24-hour Holter monitoring; echocardiography was also performed on most patients. Results: Among the 83 patients, 62 with no cardiovascular risk factors were identified. Ten of them manifested clinical or subclinical cardiac involvement: 5 reported symptoms represented mostly by frequent palpitations secondary to supraventricular arrhythmia and another 5 exhibited electrocardiographic signs of short runs of supraventricular paroxysmal tachycardia. In the absence of cardiovascular risk factors, we found symptoms or signs of heart involvement of mainly arrhythmic origin in 10 of our 83 FSHD patients (12%). Conclusions: Considering our data and those available in the literature as a whole, arrhythmic alterations seem to be detected more frequently than expected in FSHD patients.

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Journal

Journal ID (publisher-id): ENE

Journal ID (nlm-ta): Eur Neurol

Journal ID (doi): 10.1159/issn.0014-3022

Title: European Neurology

Publisher: S. Karger AG

ISSN (Print): 0014-3022

ISSN (Electronic): 1421-9913

Publication date Subscription-year: 2006

Publication date (Print): September 2006

Publication date (Electronic): 08 September 2006

Volume: 56

Issue: 1

Pages: 1-5

Affiliations

^aDepartment of Neurosciences and ^bDepartment of Internal Medicine, University of Padua, Padua, ^cDepartment of Neurological Sciences and Vision, University of Verona, Verona, ^dDepartment of Neuroscience, University of Torino, Torino, ^eInstitute of Neurology, Catholic University, Policlinico Gemelli, Roma, and ^fLaboratory of Neurogenetics, University of Modena and Reggio Emilia, Modena, Italy

Article

Publisher ID: 94248 Other ID: Eur Neurol 2006;56:1–5

DOI: 10.1159/000094248

PubMed ID: 16804309

SO-VID: 08fdbd4f-ede8-461b-b08c-4040c00f63de

License:

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

History

Date received : 18 January 2006

Date accepted : 29 March 2006

Page count

Tables: 3, References: 22, Pages: 5

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