Left ventricular noncompaction in Duchenne muscular dystrophy
Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective o...
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| Veröffentlicht in: | Journal of cardiovascular magnetic resonance 2013-08, Vol.15 (1), p.67-67, Article 67 |
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| creator | Statile, Christopher J Taylor, Michael D Mazur, Wojciech Cripe, Linda H King, Eileen Pratt, Jesse Benson, D Woodrow Hor, Kan N |
| description | Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.
Cardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF 2.3 for any segment.
LVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF 55%, 2.46 for DMD with EF |
| doi_str_mv | 10.1186/1532-429X-15-67 |
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Cardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF < 55% (n = 30), primary LVNC (n = 15) and normal controls (n = 40). The non-compacted to compacted (NC/C) ratio was measured in each of the 16 standard myocardial segments. LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.
LVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF < 55% (prevalence of 53.3%). The median maximum NC/C ratio was 1.8 for DMD with EF > 55%, 2.46 for DMD with EF < 55%, 1.54 for the normal subjects, and 3.69 for primary LVNC patients. Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.
The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.</description><identifier>ISSN: 1097-6647</identifier><identifier>ISSN: 1532-429X</identifier><identifier>EISSN: 1532-429X</identifier><identifier>DOI: 10.1186/1532-429X-15-67</identifier><identifier>PMID: 23914774</identifier><language>eng</language><publisher>England: BioMed Central Ltd</publisher><subject>Chi-Square Distribution ; Child ; Contrast Media ; Degeneration ; Development and progression ; Diagnosis ; Disorders ; Duchenne muscular dystrophy ; Female ; Gadolinium DTPA ; Genetic aspects ; Heart diseases ; Humans ; Isolated Noncompaction of the Ventricular Myocardium - complications ; Isolated Noncompaction of the Ventricular Myocardium - diagnosis ; Isolated Noncompaction of the Ventricular Myocardium - epidemiology ; Magnetic resonance ; Magnetic Resonance Imaging - methods ; Male ; Medical imaging equipment ; Medical research ; Medicine, Experimental ; Muscular dystrophy ; Muscular Dystrophy, Duchenne - complications ; Numerical control ; Patients ; Prevalence ; Segments ; Statistics, Nonparametric ; Vectorcardiography</subject><ispartof>Journal of cardiovascular magnetic resonance, 2013-08, Vol.15 (1), p.67-67, Article 67</ispartof><rights>COPYRIGHT 2013 BioMed Central Ltd.</rights><rights>2013 Statile et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</rights><rights>Copyright © 2013 Statile et al.; licensee BioMed Central Ltd. 2013 Statile et al.; licensee BioMed Central Ltd.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c552t-f6f259a12c1184c24a60370adf428efc11266e259eca8fdfd218a787084e2bfe3</citedby><cites>FETCH-LOGICAL-c552t-f6f259a12c1184c24a60370adf428efc11266e259eca8fdfd218a787084e2bfe3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3750745/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3750745/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,864,885,27924,27925,53791,53793</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23914774$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Statile, Christopher J</creatorcontrib><creatorcontrib>Taylor, Michael D</creatorcontrib><creatorcontrib>Mazur, Wojciech</creatorcontrib><creatorcontrib>Cripe, Linda H</creatorcontrib><creatorcontrib>King, Eileen</creatorcontrib><creatorcontrib>Pratt, Jesse</creatorcontrib><creatorcontrib>Benson, D Woodrow</creatorcontrib><creatorcontrib>Hor, Kan N</creatorcontrib><title>Left ventricular noncompaction in Duchenne muscular dystrophy</title><title>Journal of cardiovascular magnetic resonance</title><addtitle>J Cardiovasc Magn Reson</addtitle><description>Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.
Cardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF < 55% (n = 30), primary LVNC (n = 15) and normal controls (n = 40). The non-compacted to compacted (NC/C) ratio was measured in each of the 16 standard myocardial segments. LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.
LVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF < 55% (prevalence of 53.3%). The median maximum NC/C ratio was 1.8 for DMD with EF > 55%, 2.46 for DMD with EF < 55%, 1.54 for the normal subjects, and 3.69 for primary LVNC patients. Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.
The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.</description><subject>Chi-Square Distribution</subject><subject>Child</subject><subject>Contrast Media</subject><subject>Degeneration</subject><subject>Development and progression</subject><subject>Diagnosis</subject><subject>Disorders</subject><subject>Duchenne muscular dystrophy</subject><subject>Female</subject><subject>Gadolinium DTPA</subject><subject>Genetic aspects</subject><subject>Heart diseases</subject><subject>Humans</subject><subject>Isolated Noncompaction of the Ventricular Myocardium - complications</subject><subject>Isolated Noncompaction of the Ventricular Myocardium - diagnosis</subject><subject>Isolated Noncompaction of the Ventricular Myocardium - epidemiology</subject><subject>Magnetic resonance</subject><subject>Magnetic Resonance Imaging - methods</subject><subject>Male</subject><subject>Medical imaging equipment</subject><subject>Medical research</subject><subject>Medicine, Experimental</subject><subject>Muscular dystrophy</subject><subject>Muscular Dystrophy, Duchenne - complications</subject><subject>Numerical control</subject><subject>Patients</subject><subject>Prevalence</subject><subject>Segments</subject><subject>Statistics, Nonparametric</subject><subject>Vectorcardiography</subject><issn>1097-6647</issn><issn>1532-429X</issn><issn>1532-429X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqNks1rFTEUxYNY7Ieu3cmAIG6mzXcyCwul1lZ44EbBXUgzN52UmeQ5mSm8_94Mrz77igvJIpeT3z1cbg5Cbwk-JUTLMyIYrTltftZE1FK9QEc75WWpcaNqKbk6RMc532NMGoXVK3RIWUO4UvwIfVqBn6oHiNMY3NzbsYopujSsrZtCilWI1efZdRAjVMOct0i7ydOY1t3mNTrwts_w5vE-QT--XH2_vKlX366_Xl6saicEnWovPRWNJdSVobmj3ErMFLat51SDLyqVEgoCzmrf-pYSbZVWWHOgtx7YCTrf-q7n2wFat4xre7Mew2DHjUk2mP2XGDpzlx4MUwIrLorBx0eDMf2aIU9mCNlB39sIac6GcNYohhX-H5RyRqlmvKDvn6H3aR5j2cRCac05F_IvdWd7MCH6VEZ0i6m5EIxLLblkhTr9B1VOC0NwKYIPRd9r-PCkoQPbT11O_bx8W94Hz7agG1POI_jd3gg2S4rMkhmzZKZURqrS8e7punf8n9iw3wDWwEg</recordid><startdate>20130801</startdate><enddate>20130801</enddate><creator>Statile, Christopher J</creator><creator>Taylor, Michael D</creator><creator>Mazur, Wojciech</creator><creator>Cripe, Linda H</creator><creator>King, Eileen</creator><creator>Pratt, Jesse</creator><creator>Benson, D Woodrow</creator><creator>Hor, Kan N</creator><general>BioMed Central Ltd</general><general>BioMed Central</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>3V.</scope><scope>7SC</scope><scope>7SP</scope><scope>7U5</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8FD</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>ARAPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>JQ2</scope><scope>K9.</scope><scope>L7M</scope><scope>LK8</scope><scope>L~C</scope><scope>L~D</scope><scope>M0S</scope><scope>M1P</scope><scope>M7P</scope><scope>M7Z</scope><scope>P5Z</scope><scope>P62</scope><scope>P64</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20130801</creationdate><title>Left ventricular noncompaction in Duchenne muscular dystrophy</title><author>Statile, Christopher J ; Taylor, Michael D ; Mazur, Wojciech ; Cripe, Linda H ; King, Eileen ; Pratt, Jesse ; Benson, D Woodrow ; Hor, Kan N</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c552t-f6f259a12c1184c24a60370adf428efc11266e259eca8fdfd218a787084e2bfe3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Chi-Square Distribution</topic><topic>Child</topic><topic>Contrast Media</topic><topic>Degeneration</topic><topic>Development and progression</topic><topic>Diagnosis</topic><topic>Disorders</topic><topic>Duchenne muscular dystrophy</topic><topic>Female</topic><topic>Gadolinium DTPA</topic><topic>Genetic aspects</topic><topic>Heart diseases</topic><topic>Humans</topic><topic>Isolated Noncompaction of the Ventricular Myocardium - complications</topic><topic>Isolated Noncompaction of the Ventricular Myocardium - diagnosis</topic><topic>Isolated Noncompaction of the Ventricular Myocardium - epidemiology</topic><topic>Magnetic resonance</topic><topic>Magnetic Resonance Imaging - methods</topic><topic>Male</topic><topic>Medical imaging equipment</topic><topic>Medical research</topic><topic>Medicine, Experimental</topic><topic>Muscular dystrophy</topic><topic>Muscular Dystrophy, Duchenne - complications</topic><topic>Numerical control</topic><topic>Patients</topic><topic>Prevalence</topic><topic>Segments</topic><topic>Statistics, Nonparametric</topic><topic>Vectorcardiography</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Statile, Christopher J</creatorcontrib><creatorcontrib>Taylor, Michael D</creatorcontrib><creatorcontrib>Mazur, Wojciech</creatorcontrib><creatorcontrib>Cripe, Linda H</creatorcontrib><creatorcontrib>King, Eileen</creatorcontrib><creatorcontrib>Pratt, Jesse</creatorcontrib><creatorcontrib>Benson, D Woodrow</creatorcontrib><creatorcontrib>Hor, Kan N</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Computer and Information Systems Abstracts</collection><collection>Electronics & Communications Abstracts</collection><collection>Solid State and Superconductivity Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Technology Research Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Technology Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>Advanced Technologies & Aerospace Collection</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Technology Collection</collection><collection>Natural Science Collection</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Computer Science Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Advanced Technologies Database with Aerospace</collection><collection>ProQuest Biological Science Collection</collection><collection>Computer and Information Systems Abstracts Academic</collection><collection>Computer and Information Systems Abstracts Professional</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Biological Science Database</collection><collection>Biochemistry Abstracts 1</collection><collection>Advanced Technologies & Aerospace Database</collection><collection>ProQuest Advanced Technologies & Aerospace Collection</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Access via ProQuest (Open Access)</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Journal of cardiovascular magnetic resonance</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Statile, Christopher J</au><au>Taylor, Michael D</au><au>Mazur, Wojciech</au><au>Cripe, Linda H</au><au>King, Eileen</au><au>Pratt, Jesse</au><au>Benson, D Woodrow</au><au>Hor, Kan N</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Left ventricular noncompaction in Duchenne muscular dystrophy</atitle><jtitle>Journal of cardiovascular magnetic resonance</jtitle><addtitle>J Cardiovasc Magn Reson</addtitle><date>2013-08-01</date><risdate>2013</risdate><volume>15</volume><issue>1</issue><spage>67</spage><epage>67</epage><pages>67-67</pages><artnum>67</artnum><issn>1097-6647</issn><issn>1532-429X</issn><eissn>1532-429X</eissn><abstract>Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.
Cardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF < 55% (n = 30), primary LVNC (n = 15) and normal controls (n = 40). The non-compacted to compacted (NC/C) ratio was measured in each of the 16 standard myocardial segments. LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.
LVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF < 55% (prevalence of 53.3%). The median maximum NC/C ratio was 1.8 for DMD with EF > 55%, 2.46 for DMD with EF < 55%, 1.54 for the normal subjects, and 3.69 for primary LVNC patients. Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.
The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.</abstract><cop>England</cop><pub>BioMed Central Ltd</pub><pmid>23914774</pmid><doi>10.1186/1532-429X-15-67</doi><tpages>1</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Chi-Square Distribution Child Contrast Media Degeneration Development and progression Diagnosis Disorders Duchenne muscular dystrophy Female Gadolinium DTPA Genetic aspects Heart diseases Humans Isolated Noncompaction of the Ventricular Myocardium - complications Isolated Noncompaction of the Ventricular Myocardium - diagnosis Isolated Noncompaction of the Ventricular Myocardium - epidemiology Magnetic resonance Magnetic Resonance Imaging - methods Male Medical imaging equipment Medical research Medicine, Experimental Muscular dystrophy Muscular Dystrophy, Duchenne - complications Numerical control Patients Prevalence Segments Statistics, Nonparametric Vectorcardiography |
| title | Left ventricular noncompaction in Duchenne muscular dystrophy |
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