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Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases

There are scarce publications regarding the presentation and outcome of Becker muscular dystrophy in adulthood when idiopathic dilated cardiomyopathy is the initial disease manifestation. We performed a systematic review using Medline, Embase, Cochrane, and Scopus to identify cases of adults with id...

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Autores principales: Del Rio-Pertuz, Gaspar, Morataya, Cristina, Parmar, Kanak, Dubay, Sarah, Argueta-Sosa, Erwin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9097097/
https://www.ncbi.nlm.nih.gov/pubmed/35549971
http://dx.doi.org/10.1186/s13023-022-02346-1
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author Del Rio-Pertuz, Gaspar
Morataya, Cristina
Parmar, Kanak
Dubay, Sarah
Argueta-Sosa, Erwin
author_facet Del Rio-Pertuz, Gaspar
Morataya, Cristina
Parmar, Kanak
Dubay, Sarah
Argueta-Sosa, Erwin
author_sort Del Rio-Pertuz, Gaspar
collection PubMed
description There are scarce publications regarding the presentation and outcome of Becker muscular dystrophy in adulthood when idiopathic dilated cardiomyopathy is the initial disease manifestation. We performed a systematic review using Medline, Embase, Cochrane, and Scopus to identify cases of adults with idiopathic dilated cardiomyopathy who were subsequently diagnosed with Becker muscular dystrophy from inception through August 2020. Six cases were found. We identified young males (Median age: 26 years) with Becker muscular dystrophy who first presented with dilated cardiomyopathy. Most patients initially presented with congestive heart failure symptoms (5/6, 83%), and had a median left ventricular ejection fraction of 23%. One case did have calf pseudohypertrophy. Musculoskeletal symptoms later appeared one to six years after the initial dilated cardiomyopathy presentation. Heart transplantation was the most common management strategy (4/6, 67%). A left ventricular assist device was used in one case as a bridge to heart transplant. Dilated cardiomyopathy can be the initial presentation of Becker muscular dystrophy in the third to fourth decades of life in adult patients, and musculoskeletal symptoms can be subclinical.
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spelling pubmed-90970972022-05-13 Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases Del Rio-Pertuz, Gaspar Morataya, Cristina Parmar, Kanak Dubay, Sarah Argueta-Sosa, Erwin Orphanet J Rare Dis Review There are scarce publications regarding the presentation and outcome of Becker muscular dystrophy in adulthood when idiopathic dilated cardiomyopathy is the initial disease manifestation. We performed a systematic review using Medline, Embase, Cochrane, and Scopus to identify cases of adults with idiopathic dilated cardiomyopathy who were subsequently diagnosed with Becker muscular dystrophy from inception through August 2020. Six cases were found. We identified young males (Median age: 26 years) with Becker muscular dystrophy who first presented with dilated cardiomyopathy. Most patients initially presented with congestive heart failure symptoms (5/6, 83%), and had a median left ventricular ejection fraction of 23%. One case did have calf pseudohypertrophy. Musculoskeletal symptoms later appeared one to six years after the initial dilated cardiomyopathy presentation. Heart transplantation was the most common management strategy (4/6, 67%). A left ventricular assist device was used in one case as a bridge to heart transplant. Dilated cardiomyopathy can be the initial presentation of Becker muscular dystrophy in the third to fourth decades of life in adult patients, and musculoskeletal symptoms can be subclinical. BioMed Central 2022-05-12 /pmc/articles/PMC9097097/ /pubmed/35549971 http://dx.doi.org/10.1186/s13023-022-02346-1 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Review
Del Rio-Pertuz, Gaspar
Morataya, Cristina
Parmar, Kanak
Dubay, Sarah
Argueta-Sosa, Erwin
Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases
title Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases
title_full Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases
title_fullStr Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases
title_full_unstemmed Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases
title_short Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases
title_sort dilated cardiomyopathy as the initial presentation of becker muscular dystrophy: a systematic review of published cases
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9097097/
https://www.ncbi.nlm.nih.gov/pubmed/35549971
http://dx.doi.org/10.1186/s13023-022-02346-1
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