Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis
Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. We designed this study to determine the prevalence of left bundle branch block (LBBB), whether there is a relationship between LBBB and genetic pattern, and to assess pre...
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| creator | Fayssoil, Abdallah Ben Yaou, Rabah Ogna, Adam Chaffaut, Cendrine Leturcq, France Nardi, Olivier Wahbi, Karim Duboc, Denis Lofaso, Frederic Prigent, Helene Clair, Bernard Crenn, Pascal Nicolas, Guillaume Laforet, Pascal Behin, Anthony Chevret, Sylvie Orlikowski, David Annane, Djillali |
| description | Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. We designed this study to determine the prevalence of left bundle branch block (LBBB), whether there is a relationship between LBBB and genetic pattern, and to assess predictive factors for acute cardiac events and mortality in adult DMD patients.
We reviewed the charts of DMD followed at the Home Mechanical Ventilation Unit of the Raymond Poincare University Hospital.
A total of 121 patients, aged from 18 to 41 years have been included in our study. Median vital capacity (VC) was 12% [7; 19.5] of predicted. Almost all patients were on home mechanical ventilation (95%). LBBB was present in 15 patients (13%); among them, 10 disclosed exonic deletions. After a median follow up of 6 years, 21 patients (17%) experienced acute heart failure (AHF), 7 patients (6%) supraventricular arrhythmia, 3 patients (2.4%) ventricular tachycardia, 4 patients (3%) significant electrical disturbances. LBBB was significantly associated with cardiac events (OR = 12.7; 95%CI [3.78-42.7]; p |
| doi_str_mv | 10.1371/journal.pone.0190518 |
| format | Article |
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We reviewed the charts of DMD followed at the Home Mechanical Ventilation Unit of the Raymond Poincare University Hospital.
A total of 121 patients, aged from 18 to 41 years have been included in our study. Median vital capacity (VC) was 12% [7; 19.5] of predicted. Almost all patients were on home mechanical ventilation (95%). LBBB was present in 15 patients (13%); among them, 10 disclosed exonic deletions. After a median follow up of 6 years, 21 patients (17%) experienced acute heart failure (AHF), 7 patients (6%) supraventricular arrhythmia, 3 patients (2.4%) ventricular tachycardia, 4 patients (3%) significant electrical disturbances. LBBB was significantly associated with cardiac events (OR = 12.7; 95%CI [3.78-42.7]; p <0.0001) and mortality (OR = 4.4; 95%CI [1.44-13.7]; p 0.009). Presence of residual dystrophin protein was not associated with significant less cardiac events. Age and LVEF were also predictive factors for cardiac events and mortality.
LBBB is relatively frequent in DMD and is a major predictive factor for cardiac events and mortality. Presence of residual dystrophin protein was not associated with a lower incidence of cardiac events.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0190518</identifier><identifier>PMID: 29304097</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Arrhythmia ; Biology and Life Sciences ; Cardiac function ; Chromosomes ; Congestive heart failure ; Duchenne's muscular dystrophy ; Dystrophin ; Dystrophy ; Electrocardiography ; Genetic relationship ; Heart failure ; Life Sciences ; Mechanical ventilation ; Medical prognosis ; Medicine and Health Sciences ; Mortality ; Muscular dystrophy ; Mutation ; Patients ; Proteins ; Tachycardia ; Ventilation ; Ventricle</subject><ispartof>PloS one, 2018-01, Vol.13 (1), p.e0190518-e0190518</ispartof><rights>2018 Fayssoil et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><rights>2018 Fayssoil et al 2018 Fayssoil et al</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c560t-6704cf1e3f82fd6b4f539f173e53e9031c690715f8bcbb08895a14c9be8b23e3</citedby><cites>FETCH-LOGICAL-c560t-6704cf1e3f82fd6b4f539f173e53e9031c690715f8bcbb08895a14c9be8b23e3</cites><orcidid>0000-0003-3844-5438 ; 0000-0001-6805-8944 ; 0000-0002-0492-3414</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5755816/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5755816/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,315,729,782,786,866,887,2104,2930,23873,27931,27932,53798,53800</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29304097$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://hal.science/hal-04015387$$DView record in HAL$$Hfree_for_read</backlink></links><search><contributor>Fraidenraich, Diego</contributor><creatorcontrib>Fayssoil, Abdallah</creatorcontrib><creatorcontrib>Ben Yaou, Rabah</creatorcontrib><creatorcontrib>Ogna, Adam</creatorcontrib><creatorcontrib>Chaffaut, Cendrine</creatorcontrib><creatorcontrib>Leturcq, France</creatorcontrib><creatorcontrib>Nardi, Olivier</creatorcontrib><creatorcontrib>Wahbi, Karim</creatorcontrib><creatorcontrib>Duboc, Denis</creatorcontrib><creatorcontrib>Lofaso, Frederic</creatorcontrib><creatorcontrib>Prigent, Helene</creatorcontrib><creatorcontrib>Clair, Bernard</creatorcontrib><creatorcontrib>Crenn, Pascal</creatorcontrib><creatorcontrib>Nicolas, Guillaume</creatorcontrib><creatorcontrib>Laforet, Pascal</creatorcontrib><creatorcontrib>Behin, Anthony</creatorcontrib><creatorcontrib>Chevret, Sylvie</creatorcontrib><creatorcontrib>Orlikowski, David</creatorcontrib><creatorcontrib>Annane, Djillali</creatorcontrib><title>Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis</title><title>PloS one</title><addtitle>PLoS One</addtitle><description>Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. We designed this study to determine the prevalence of left bundle branch block (LBBB), whether there is a relationship between LBBB and genetic pattern, and to assess predictive factors for acute cardiac events and mortality in adult DMD patients.
We reviewed the charts of DMD followed at the Home Mechanical Ventilation Unit of the Raymond Poincare University Hospital.
A total of 121 patients, aged from 18 to 41 years have been included in our study. Median vital capacity (VC) was 12% [7; 19.5] of predicted. Almost all patients were on home mechanical ventilation (95%). LBBB was present in 15 patients (13%); among them, 10 disclosed exonic deletions. After a median follow up of 6 years, 21 patients (17%) experienced acute heart failure (AHF), 7 patients (6%) supraventricular arrhythmia, 3 patients (2.4%) ventricular tachycardia, 4 patients (3%) significant electrical disturbances. LBBB was significantly associated with cardiac events (OR = 12.7; 95%CI [3.78-42.7]; p <0.0001) and mortality (OR = 4.4; 95%CI [1.44-13.7]; p 0.009). Presence of residual dystrophin protein was not associated with significant less cardiac events. Age and LVEF were also predictive factors for cardiac events and mortality.
LBBB is relatively frequent in DMD and is a major predictive factor for cardiac events and mortality. Presence of residual dystrophin protein was not associated with a lower incidence of cardiac events.</description><subject>Arrhythmia</subject><subject>Biology and Life Sciences</subject><subject>Cardiac function</subject><subject>Chromosomes</subject><subject>Congestive heart failure</subject><subject>Duchenne's muscular dystrophy</subject><subject>Dystrophin</subject><subject>Dystrophy</subject><subject>Electrocardiography</subject><subject>Genetic relationship</subject><subject>Heart failure</subject><subject>Life Sciences</subject><subject>Mechanical ventilation</subject><subject>Medical prognosis</subject><subject>Medicine and Health Sciences</subject><subject>Mortality</subject><subject>Muscular dystrophy</subject><subject>Mutation</subject><subject>Patients</subject><subject>Proteins</subject><subject>Tachycardia</subject><subject>Ventilation</subject><subject>Ventricle</subject><issn>1932-6203</issn><issn>1932-6203</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>DOA</sourceid><recordid>eNptUl1v0zAUjRCIjcI_QGCJF5BosePYjnlAmsbHJlWCh71btnPdpLh2sJNK_fekNJu2iSdb1-ec63vuKYrXBK8IFeTTNo4paL_qY4AVJhIzUj8pzomk5ZKXmD69dz8rXuS8xZjRmvPnxVkpKa6wFOfFZg1uQGYMjQdkkg62RcZH-xt1AX0dbQshANqN2Y5eJ9Qc8pBi3x4-o18J9tpDsPARbSDA0FmUwOuhiyG3XY90aFCf4ibE3OWXxTOnfYZX87kobr5_u7m8Wq5__ri-vFgvLeN4WHKBK-sIUFeXruGmcoxKRwQFRkFiSiyXWBDmamONwXUtmSaVlQZqU1Kgi-LtSbb3MavZoqxKKjGvBJ8MWBTXJ0QT9Vb1qdvpdFBRd-pfIaaN0mmaxYMyQtZcU6IF5ZWpuAHBKs6YK0FL7OpJ68vcbTQ7aCyEIWn_QPThS-hatYl7xQRjNeGTwIeTQPuIdnWxVsfatCUyLU3syYR9PzdL8c8IeVC7LlvwXgeIY1ZETmbQacYj9N0j6P-dqE4om2LOCdzdDwhWx4jdstQxYmqO2ER7c3_oO9Jtpuhf6inPWA</recordid><startdate>20180101</startdate><enddate>20180101</enddate><creator>Fayssoil, 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bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis</title><author>Fayssoil, Abdallah ; Ben Yaou, Rabah ; Ogna, Adam ; Chaffaut, Cendrine ; Leturcq, France ; Nardi, Olivier ; Wahbi, Karim ; Duboc, Denis ; Lofaso, Frederic ; Prigent, Helene ; Clair, Bernard ; Crenn, Pascal ; Nicolas, Guillaume ; Laforet, Pascal ; Behin, Anthony ; Chevret, Sylvie ; Orlikowski, David ; Annane, Djillali</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c560t-6704cf1e3f82fd6b4f539f173e53e9031c690715f8bcbb08895a14c9be8b23e3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Arrhythmia</topic><topic>Biology and Life Sciences</topic><topic>Cardiac function</topic><topic>Chromosomes</topic><topic>Congestive heart failure</topic><topic>Duchenne's muscular dystrophy</topic><topic>Dystrophin</topic><topic>Dystrophy</topic><topic>Electrocardiography</topic><topic>Genetic relationship</topic><topic>Heart failure</topic><topic>Life Sciences</topic><topic>Mechanical ventilation</topic><topic>Medical prognosis</topic><topic>Medicine and Health Sciences</topic><topic>Mortality</topic><topic>Muscular dystrophy</topic><topic>Mutation</topic><topic>Patients</topic><topic>Proteins</topic><topic>Tachycardia</topic><topic>Ventilation</topic><topic>Ventricle</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Fayssoil, Abdallah</creatorcontrib><creatorcontrib>Ben Yaou, Rabah</creatorcontrib><creatorcontrib>Ogna, Adam</creatorcontrib><creatorcontrib>Chaffaut, Cendrine</creatorcontrib><creatorcontrib>Leturcq, France</creatorcontrib><creatorcontrib>Nardi, Olivier</creatorcontrib><creatorcontrib>Wahbi, Karim</creatorcontrib><creatorcontrib>Duboc, Denis</creatorcontrib><creatorcontrib>Lofaso, 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Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Fayssoil, Abdallah</au><au>Ben Yaou, Rabah</au><au>Ogna, Adam</au><au>Chaffaut, Cendrine</au><au>Leturcq, France</au><au>Nardi, Olivier</au><au>Wahbi, Karim</au><au>Duboc, Denis</au><au>Lofaso, Frederic</au><au>Prigent, Helene</au><au>Clair, Bernard</au><au>Crenn, Pascal</au><au>Nicolas, Guillaume</au><au>Laforet, Pascal</au><au>Behin, Anthony</au><au>Chevret, Sylvie</au><au>Orlikowski, David</au><au>Annane, Djillali</au><au>Fraidenraich, Diego</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis</atitle><jtitle>PloS one</jtitle><addtitle>PLoS One</addtitle><date>2018-01-01</date><risdate>2018</risdate><volume>13</volume><issue>1</issue><spage>e0190518</spage><epage>e0190518</epage><pages>e0190518-e0190518</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><abstract>Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. We designed this study to determine the prevalence of left bundle branch block (LBBB), whether there is a relationship between LBBB and genetic pattern, and to assess predictive factors for acute cardiac events and mortality in adult DMD patients.
We reviewed the charts of DMD followed at the Home Mechanical Ventilation Unit of the Raymond Poincare University Hospital.
A total of 121 patients, aged from 18 to 41 years have been included in our study. Median vital capacity (VC) was 12% [7; 19.5] of predicted. Almost all patients were on home mechanical ventilation (95%). LBBB was present in 15 patients (13%); among them, 10 disclosed exonic deletions. After a median follow up of 6 years, 21 patients (17%) experienced acute heart failure (AHF), 7 patients (6%) supraventricular arrhythmia, 3 patients (2.4%) ventricular tachycardia, 4 patients (3%) significant electrical disturbances. LBBB was significantly associated with cardiac events (OR = 12.7; 95%CI [3.78-42.7]; p <0.0001) and mortality (OR = 4.4; 95%CI [1.44-13.7]; p 0.009). Presence of residual dystrophin protein was not associated with significant less cardiac events. Age and LVEF were also predictive factors for cardiac events and mortality.
LBBB is relatively frequent in DMD and is a major predictive factor for cardiac events and mortality. Presence of residual dystrophin protein was not associated with a lower incidence of cardiac events.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>29304097</pmid><doi>10.1371/journal.pone.0190518</doi><orcidid>https://orcid.org/0000-0003-3844-5438</orcidid><orcidid>https://orcid.org/0000-0001-6805-8944</orcidid><orcidid>https://orcid.org/0000-0002-0492-3414</orcidid><oa>free_for_read</oa></addata></record> |
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| identifier | ISSN: 1932-6203 |
| ispartof | PloS one, 2018-01, Vol.13 (1), p.e0190518-e0190518 |
| issn | 1932-6203 1932-6203 |
| language | eng |
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| source | DOAJ Directory of Open Access Journals; Public Library of Science (PLoS) Journals Open Access; EZB-FREE-00999 freely available EZB journals; PubMed Central; Free Full-Text Journals in Chemistry |
| subjects | Arrhythmia Biology and Life Sciences Cardiac function Chromosomes Congestive heart failure Duchenne's muscular dystrophy Dystrophin Dystrophy Electrocardiography Genetic relationship Heart failure Life Sciences Mechanical ventilation Medical prognosis Medicine and Health Sciences Mortality Muscular dystrophy Mutation Patients Proteins Tachycardia Ventilation Ventricle |
| title | Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-03T23%3A36%3A57IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_plos_&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Left%20bundle%20branch%20block%20in%20Duchenne%20muscular%20dystrophy:%20Prevalence,%20genetic%20relationship%20and%20prognosis&rft.jtitle=PloS%20one&rft.au=Fayssoil,%20Abdallah&rft.date=2018-01-01&rft.volume=13&rft.issue=1&rft.spage=e0190518&rft.epage=e0190518&rft.pages=e0190518-e0190518&rft.issn=1932-6203&rft.eissn=1932-6203&rft_id=info:doi/10.1371/journal.pone.0190518&rft_dat=%3Cproquest_plos_%3E2390647605%3C/proquest_plos_%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2390647605&rft_id=info:pmid/29304097&rft_doaj_id=oai_doaj_org_article_b7986a31a7364b46be754655f2ea90f8&rfr_iscdi=true |