Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy
The relationship between pulmonary function and right ventricle (RV) in Duchenne muscular dystrophy (DMD) has not been evaluated. Using cardiac magnetic resonance (CMR), we describe the relationship of RV size and function with spirometry in a DMD cohort. Fifty-seven boys undergoing CMR and pulmonar...
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| Veröffentlicht in: | Pediatric cardiology 2016-06, Vol.37 (5), p.878-883 |
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| creator | Mehmood, Muddassir Ambach, Stephanie A. Taylor, Michael D. Jefferies, John L. Raman, Subha V. Taylor, Robin J. Sawani, Hemant Mathew, Jacob Mazur, Wojciech Hor, Kan N. Al-Khalidi, Hussein R. |
| description | The relationship between pulmonary function and right ventricle (RV) in Duchenne muscular dystrophy (DMD) has not been evaluated. Using cardiac magnetic resonance (CMR), we describe the relationship of RV size and function with spirometry in a DMD cohort. Fifty-seven boys undergoing CMR and pulmonary function testing within 1 month at a single center (2013–2015) were enrolled. Comparisons of RV ejection fraction (RVEF) and end-diastolic volume index (RVEDVI) were made across categories of percent forced vital capacity (FVC%), and relationships were assessed. Mean age was 15.5 ± 3.5 years. Spirometry and CMR were performed within 3.9 ± 4.1 days. Median FVC% was 92.0 % (67.5–116.5 %). Twenty-three (40 %) patients had abnormal FVC% ( |
| doi_str_mv | 10.1007/s00246-016-1362-2 |
| format | Article |
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r
= 0.31,
p
= 0.02 and
r
= 0.39,
p
= 0.003, respectively). In a large DMD cohort, RVEF and RVEDVI were related to FVC%. Worsening respiratory status may guide monitoring of cardiac function in these patients.</description><identifier>ISSN: 0172-0643</identifier><identifier>EISSN: 1432-1971</identifier><identifier>DOI: 10.1007/s00246-016-1362-2</identifier><identifier>PMID: 26936620</identifier><language>eng</language><publisher>New York: Springer US</publisher><subject>Adolescent ; Cardiac Surgery ; Cardiology ; Child ; Duchenne muscular dystrophy ; Heart ; Heart Ventricles ; Humans ; Male ; Medicine ; Medicine & Public Health ; Muscular Dystrophy, Duchenne ; Original Article ; Pulmonary function tests ; Respiratory Function Tests ; Stroke Volume ; Vascular Surgery ; Ventricular Function, Right ; Young Adult</subject><ispartof>Pediatric cardiology, 2016-06, Vol.37 (5), p.878-883</ispartof><rights>Springer Science+Business Media New York 2016</rights><rights>COPYRIGHT 2016 Springer</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c411t-22c8fc302aa554c38bc1c445799c7e6295d167349f11b4a9bdd5d7a86632f8d73</citedby><cites>FETCH-LOGICAL-c411t-22c8fc302aa554c38bc1c445799c7e6295d167349f11b4a9bdd5d7a86632f8d73</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00246-016-1362-2$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00246-016-1362-2$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>315,782,786,27933,27934,41497,42566,51328</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26936620$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Mehmood, Muddassir</creatorcontrib><creatorcontrib>Ambach, Stephanie A.</creatorcontrib><creatorcontrib>Taylor, Michael D.</creatorcontrib><creatorcontrib>Jefferies, John L.</creatorcontrib><creatorcontrib>Raman, Subha V.</creatorcontrib><creatorcontrib>Taylor, Robin J.</creatorcontrib><creatorcontrib>Sawani, Hemant</creatorcontrib><creatorcontrib>Mathew, Jacob</creatorcontrib><creatorcontrib>Mazur, Wojciech</creatorcontrib><creatorcontrib>Hor, Kan N.</creatorcontrib><creatorcontrib>Al-Khalidi, Hussein R.</creatorcontrib><title>Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy</title><title>Pediatric cardiology</title><addtitle>Pediatr Cardiol</addtitle><addtitle>Pediatr Cardiol</addtitle><description>The relationship between pulmonary function and right ventricle (RV) in Duchenne muscular dystrophy (DMD) has not been evaluated. Using cardiac magnetic resonance (CMR), we describe the relationship of RV size and function with spirometry in a DMD cohort. Fifty-seven boys undergoing CMR and pulmonary function testing within 1 month at a single center (2013–2015) were enrolled. Comparisons of RV ejection fraction (RVEF) and end-diastolic volume index (RVEDVI) were made across categories of percent forced vital capacity (FVC%), and relationships were assessed. Mean age was 15.5 ± 3.5 years. Spirometry and CMR were performed within 3.9 ± 4.1 days. Median FVC% was 92.0 % (67.5–116.5 %). Twenty-three (40 %) patients had abnormal FVC% (<80 %) of which 13 (57 %) had mild (FVC% 60–79 %), 6 (26 %) had moderate (FVC% 40–59 %), and 4 (17 %) had severe (FVC <40 %) reductions. Mean RVEF was 58.3 ± 3.7 %. Patients with abnormal FVC% were older and had lower RVEF and RVEDVI. Both RVEF and RVEDVI were significantly associated with FVC% (
r
= 0.31,
p
= 0.02 and
r
= 0.39,
p
= 0.003, respectively). In a large DMD cohort, RVEF and RVEDVI were related to FVC%. Worsening respiratory status may guide monitoring of cardiac function in these patients.</description><subject>Adolescent</subject><subject>Cardiac Surgery</subject><subject>Cardiology</subject><subject>Child</subject><subject>Duchenne muscular dystrophy</subject><subject>Heart</subject><subject>Heart Ventricles</subject><subject>Humans</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Muscular Dystrophy, Duchenne</subject><subject>Original Article</subject><subject>Pulmonary function tests</subject><subject>Respiratory Function Tests</subject><subject>Stroke Volume</subject><subject>Vascular Surgery</subject><subject>Ventricular Function, Right</subject><subject>Young Adult</subject><issn>0172-0643</issn><issn>1432-1971</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kV1rFDEUhoModq3-AG8k4I03U_OdmcvS2ipUhK16G7KZzE6W2WTMB7L--maYKggiuTiQ87yHw3kAeI3RBUZIvk8IESYahEWDqSANeQI2mFHS4E7ip2CDsCQNEoyegRcpHRBCLWr5c3BGREeFIGgDDls76eyCT6ObYRjg1u3HDL9bn6MzZdIR3rtfFmrfw5vizYLCny6PcGvT7KLOIZ7gfda5JOg8vC5mtN5b-LmkNX59SjmGeTy9BM8GPSX76rGeg283H75efWzuvtx-urq8awzDODeEmHYwFBGtOWeGtjuDDWNcdp2RVpCO91hIyroB4x3T3a7veS91KwQlQ9tLeg7erXPnGH4Um7I6umTsNGlvQ0kKy463kggmKvp2Rfd6ssr5IeSozYKrS4k5r3cUC3XxD6q-3h6dCd4Orv7_FcBrwMSQUrSDmqM76nhSGKnFnFrNqWpOLeYUqZk3j1uX3dH2fxK_VVWArECqLb-3UR1Cib5e8j9THwBHoaLo</recordid><startdate>20160601</startdate><enddate>20160601</enddate><creator>Mehmood, Muddassir</creator><creator>Ambach, Stephanie A.</creator><creator>Taylor, Michael D.</creator><creator>Jefferies, John L.</creator><creator>Raman, Subha V.</creator><creator>Taylor, Robin J.</creator><creator>Sawani, Hemant</creator><creator>Mathew, Jacob</creator><creator>Mazur, Wojciech</creator><creator>Hor, Kan N.</creator><creator>Al-Khalidi, Hussein R.</creator><general>Springer US</general><general>Springer</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20160601</creationdate><title>Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy</title><author>Mehmood, Muddassir ; Ambach, Stephanie A. ; Taylor, Michael D. ; Jefferies, John L. ; Raman, Subha V. ; Taylor, Robin J. ; Sawani, Hemant ; Mathew, Jacob ; Mazur, Wojciech ; Hor, Kan N. ; Al-Khalidi, Hussein R.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c411t-22c8fc302aa554c38bc1c445799c7e6295d167349f11b4a9bdd5d7a86632f8d73</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2016</creationdate><topic>Adolescent</topic><topic>Cardiac Surgery</topic><topic>Cardiology</topic><topic>Child</topic><topic>Duchenne muscular dystrophy</topic><topic>Heart</topic><topic>Heart Ventricles</topic><topic>Humans</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Muscular Dystrophy, Duchenne</topic><topic>Original Article</topic><topic>Pulmonary function tests</topic><topic>Respiratory Function Tests</topic><topic>Stroke Volume</topic><topic>Vascular Surgery</topic><topic>Ventricular Function, Right</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Mehmood, Muddassir</creatorcontrib><creatorcontrib>Ambach, Stephanie A.</creatorcontrib><creatorcontrib>Taylor, Michael D.</creatorcontrib><creatorcontrib>Jefferies, John L.</creatorcontrib><creatorcontrib>Raman, Subha V.</creatorcontrib><creatorcontrib>Taylor, Robin J.</creatorcontrib><creatorcontrib>Sawani, Hemant</creatorcontrib><creatorcontrib>Mathew, Jacob</creatorcontrib><creatorcontrib>Mazur, Wojciech</creatorcontrib><creatorcontrib>Hor, Kan N.</creatorcontrib><creatorcontrib>Al-Khalidi, Hussein R.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric cardiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Mehmood, Muddassir</au><au>Ambach, Stephanie A.</au><au>Taylor, Michael D.</au><au>Jefferies, John L.</au><au>Raman, Subha V.</au><au>Taylor, Robin J.</au><au>Sawani, Hemant</au><au>Mathew, Jacob</au><au>Mazur, Wojciech</au><au>Hor, Kan N.</au><au>Al-Khalidi, Hussein R.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy</atitle><jtitle>Pediatric cardiology</jtitle><stitle>Pediatr Cardiol</stitle><addtitle>Pediatr Cardiol</addtitle><date>2016-06-01</date><risdate>2016</risdate><volume>37</volume><issue>5</issue><spage>878</spage><epage>883</epage><pages>878-883</pages><issn>0172-0643</issn><eissn>1432-1971</eissn><abstract>The relationship between pulmonary function and right ventricle (RV) in Duchenne muscular dystrophy (DMD) has not been evaluated. Using cardiac magnetic resonance (CMR), we describe the relationship of RV size and function with spirometry in a DMD cohort. Fifty-seven boys undergoing CMR and pulmonary function testing within 1 month at a single center (2013–2015) were enrolled. Comparisons of RV ejection fraction (RVEF) and end-diastolic volume index (RVEDVI) were made across categories of percent forced vital capacity (FVC%), and relationships were assessed. Mean age was 15.5 ± 3.5 years. Spirometry and CMR were performed within 3.9 ± 4.1 days. Median FVC% was 92.0 % (67.5–116.5 %). Twenty-three (40 %) patients had abnormal FVC% (<80 %) of which 13 (57 %) had mild (FVC% 60–79 %), 6 (26 %) had moderate (FVC% 40–59 %), and 4 (17 %) had severe (FVC <40 %) reductions. Mean RVEF was 58.3 ± 3.7 %. Patients with abnormal FVC% were older and had lower RVEF and RVEDVI. Both RVEF and RVEDVI were significantly associated with FVC% (
r
= 0.31,
p
= 0.02 and
r
= 0.39,
p
= 0.003, respectively). In a large DMD cohort, RVEF and RVEDVI were related to FVC%. Worsening respiratory status may guide monitoring of cardiac function in these patients.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>26936620</pmid><doi>10.1007/s00246-016-1362-2</doi><tpages>6</tpages></addata></record> |
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| source | MEDLINE; SpringerNature Journals |
| subjects | Adolescent Cardiac Surgery Cardiology Child Duchenne muscular dystrophy Heart Heart Ventricles Humans Male Medicine Medicine & Public Health Muscular Dystrophy, Duchenne Original Article Pulmonary function tests Respiratory Function Tests Stroke Volume Vascular Surgery Ventricular Function, Right Young Adult |
| title | Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy |
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