Evaluation of cardiac findings in mucopolysaccharidosis
Purpose Mucopolysaccharidoses (MPS) are a group of rare genetic diseases and heart involvement is one of the important conflicts in most types, which may cause serious complications. We used M-Mode and two-dimensional speckle tracking echocardiography (2D-STE) to explore cardiovascular involvements...
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| Veröffentlicht in: | The international journal of cardiovascular imaging 2024-01, Vol.40 (1), p.73-78 |
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| container_title | The international journal of cardiovascular imaging |
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| creator | Dehghan, Bahar Rostampour, Noushin Sedighi, Mohsen Saryazdi, Mohammad Hosseini Rizi, Mohammad Jokar Mostofizadeh, Neda Hashemipour, Mahin Khoshhali, Mehri |
| description | Purpose
Mucopolysaccharidoses (MPS) are a group of rare genetic diseases and heart involvement is one of the important conflicts in most types, which may cause serious complications. We used M-Mode and two-dimensional speckle tracking echocardiography (2D-STE) to explore cardiovascular involvements in MPS patients.
Method
The present cross-sectional study investigated the frequency of cardiac involvements in MPS patients. Included participants were MPS types I, II, III, IV, and VI who underwent specialized echocardiography exams to assess valvular function, systolic and diastolic function, left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS).
Results
35 patients were enrolled in this study. The total mean age of patients was 9.58 ± 5.11 years and 71.4% were male. Type IV (40%) and type III (31.4%) were the most frequent MPS. Although LVEF did not differ notably among MPS types, GLS was significantly different (p = 0.029). Mitral regurgitation was observed remarkably more in MPS type III (p = 0.001) while mitral stenosis was more common in type III (p = 0.007). There was a significant association between LVEF and GLS (β= -0.662; p = 0.025) and between LVEF and MPS type (β = 1.82; p = 0.025) when adjusted for GLS.
Conclusion
Cardiac complications are very common and are one of the most important causes of death in MPS patients. 2D-STE seems to be superior to M-Mode for detection of early and subclinical cardiac dysfunction in MPS patients. |
| doi_str_mv | 10.1007/s10554-023-02983-y |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2878290189</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2911622885</sourcerecordid><originalsourceid>FETCH-LOGICAL-c326t-d08ca90abedff8efa01dc13ed1adf39e492514cbb9cfddf6771aee265a4cdddd3</originalsourceid><addsrcrecordid>eNp9kMtKxDAUhoMozjj6Ai6k4MZNNZe2SZYyjBcYcKPrkOYyZmibMZkKffuJdrzgwsAhgfOd_4QPgHMErxGE9CYiWJZFDjFJxRnJhwMwRYyWOSMIH_56T8BJjGsIESWcH4MJoawoC8ingC7eZdPLrfNd5m2mZNBOqsy6TrtuFTPXZW2v_MY3Q5RKvcrgtI8unoIjK5tozvb3DLzcLZ7nD_ny6f5xfrvMFcHVNteQKcmhrI22lhkrIdIKEaOR1JZwU3BcokLVNVdWa1tRiqQxuCploXQ6ZAauxtxN8G-9iVvRuqhM08jO-D4KzCjDHCLGE3r5B137PnTpdwJzhCqMGSsThUdKBR9jMFZsgmtlGASC4kOrGLWKpFV8ahVDGrrYR_d1a_T3yJfHBJARiKnVrUz42f1P7A4wJoTO</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2911622885</pqid></control><display><type>article</type><title>Evaluation of cardiac findings in mucopolysaccharidosis</title><source>SpringerLink Journals</source><creator>Dehghan, Bahar ; Rostampour, Noushin ; Sedighi, Mohsen ; Saryazdi, Mohammad Hosseini ; Rizi, Mohammad Jokar ; Mostofizadeh, Neda ; Hashemipour, Mahin ; Khoshhali, Mehri</creator><creatorcontrib>Dehghan, Bahar ; Rostampour, Noushin ; Sedighi, Mohsen ; Saryazdi, Mohammad Hosseini ; Rizi, Mohammad Jokar ; Mostofizadeh, Neda ; Hashemipour, Mahin ; Khoshhali, Mehri</creatorcontrib><description>Purpose
Mucopolysaccharidoses (MPS) are a group of rare genetic diseases and heart involvement is one of the important conflicts in most types, which may cause serious complications. We used M-Mode and two-dimensional speckle tracking echocardiography (2D-STE) to explore cardiovascular involvements in MPS patients.
Method
The present cross-sectional study investigated the frequency of cardiac involvements in MPS patients. Included participants were MPS types I, II, III, IV, and VI who underwent specialized echocardiography exams to assess valvular function, systolic and diastolic function, left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS).
Results
35 patients were enrolled in this study. The total mean age of patients was 9.58 ± 5.11 years and 71.4% were male. Type IV (40%) and type III (31.4%) were the most frequent MPS. Although LVEF did not differ notably among MPS types, GLS was significantly different (p = 0.029). Mitral regurgitation was observed remarkably more in MPS type III (p = 0.001) while mitral stenosis was more common in type III (p = 0.007). There was a significant association between LVEF and GLS (β= -0.662; p = 0.025) and between LVEF and MPS type (β = 1.82; p = 0.025) when adjusted for GLS.
Conclusion
Cardiac complications are very common and are one of the most important causes of death in MPS patients. 2D-STE seems to be superior to M-Mode for detection of early and subclinical cardiac dysfunction in MPS patients.</description><identifier>ISSN: 1875-8312</identifier><identifier>ISSN: 1569-5794</identifier><identifier>EISSN: 1875-8312</identifier><identifier>EISSN: 1573-0743</identifier><identifier>DOI: 10.1007/s10554-023-02983-y</identifier><identifier>PMID: 37845409</identifier><language>eng</language><publisher>Dordrecht: Springer Netherlands</publisher><subject>Age ; Blood pressure ; Body mass index ; Cardiac Imaging ; Cardiology ; Complications ; Congenital diseases ; Cross-sectional studies ; Echocardiography ; Enzymes ; Genetic disorders ; Heart ; Heart diseases ; Imaging ; Medicine ; Medicine & Public Health ; Metabolic disorders ; Mucopolysaccharidosis ; Original Paper ; Pulmonary arteries ; Radiology ; Regurgitation ; Research centers ; Stenosis ; Variance analysis ; Ventricle</subject><ispartof>The international journal of cardiovascular imaging, 2024-01, Vol.40 (1), p.73-78</ispartof><rights>The Author(s), under exclusive licence to Springer Nature B.V. 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.</rights><rights>2023. The Author(s), under exclusive licence to Springer Nature B.V.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c326t-d08ca90abedff8efa01dc13ed1adf39e492514cbb9cfddf6771aee265a4cdddd3</cites><orcidid>0000-0003-3684-8196</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s10554-023-02983-y$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s10554-023-02983-y$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,776,780,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37845409$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Dehghan, Bahar</creatorcontrib><creatorcontrib>Rostampour, Noushin</creatorcontrib><creatorcontrib>Sedighi, Mohsen</creatorcontrib><creatorcontrib>Saryazdi, Mohammad Hosseini</creatorcontrib><creatorcontrib>Rizi, Mohammad Jokar</creatorcontrib><creatorcontrib>Mostofizadeh, Neda</creatorcontrib><creatorcontrib>Hashemipour, Mahin</creatorcontrib><creatorcontrib>Khoshhali, Mehri</creatorcontrib><title>Evaluation of cardiac findings in mucopolysaccharidosis</title><title>The international journal of cardiovascular imaging</title><addtitle>Int J Cardiovasc Imaging</addtitle><addtitle>Int J Cardiovasc Imaging</addtitle><description>Purpose
Mucopolysaccharidoses (MPS) are a group of rare genetic diseases and heart involvement is one of the important conflicts in most types, which may cause serious complications. We used M-Mode and two-dimensional speckle tracking echocardiography (2D-STE) to explore cardiovascular involvements in MPS patients.
Method
The present cross-sectional study investigated the frequency of cardiac involvements in MPS patients. Included participants were MPS types I, II, III, IV, and VI who underwent specialized echocardiography exams to assess valvular function, systolic and diastolic function, left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS).
Results
35 patients were enrolled in this study. The total mean age of patients was 9.58 ± 5.11 years and 71.4% were male. Type IV (40%) and type III (31.4%) were the most frequent MPS. Although LVEF did not differ notably among MPS types, GLS was significantly different (p = 0.029). Mitral regurgitation was observed remarkably more in MPS type III (p = 0.001) while mitral stenosis was more common in type III (p = 0.007). There was a significant association between LVEF and GLS (β= -0.662; p = 0.025) and between LVEF and MPS type (β = 1.82; p = 0.025) when adjusted for GLS.
Conclusion
Cardiac complications are very common and are one of the most important causes of death in MPS patients. 2D-STE seems to be superior to M-Mode for detection of early and subclinical cardiac dysfunction in MPS patients.</description><subject>Age</subject><subject>Blood pressure</subject><subject>Body mass index</subject><subject>Cardiac Imaging</subject><subject>Cardiology</subject><subject>Complications</subject><subject>Congenital diseases</subject><subject>Cross-sectional studies</subject><subject>Echocardiography</subject><subject>Enzymes</subject><subject>Genetic disorders</subject><subject>Heart</subject><subject>Heart diseases</subject><subject>Imaging</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Metabolic disorders</subject><subject>Mucopolysaccharidosis</subject><subject>Original Paper</subject><subject>Pulmonary arteries</subject><subject>Radiology</subject><subject>Regurgitation</subject><subject>Research centers</subject><subject>Stenosis</subject><subject>Variance analysis</subject><subject>Ventricle</subject><issn>1875-8312</issn><issn>1569-5794</issn><issn>1875-8312</issn><issn>1573-0743</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><sourceid>BENPR</sourceid><recordid>eNp9kMtKxDAUhoMozjj6Ai6k4MZNNZe2SZYyjBcYcKPrkOYyZmibMZkKffuJdrzgwsAhgfOd_4QPgHMErxGE9CYiWJZFDjFJxRnJhwMwRYyWOSMIH_56T8BJjGsIESWcH4MJoawoC8ingC7eZdPLrfNd5m2mZNBOqsy6TrtuFTPXZW2v_MY3Q5RKvcrgtI8unoIjK5tozvb3DLzcLZ7nD_ny6f5xfrvMFcHVNteQKcmhrI22lhkrIdIKEaOR1JZwU3BcokLVNVdWa1tRiqQxuCploXQ6ZAauxtxN8G-9iVvRuqhM08jO-D4KzCjDHCLGE3r5B137PnTpdwJzhCqMGSsThUdKBR9jMFZsgmtlGASC4kOrGLWKpFV8ahVDGrrYR_d1a_T3yJfHBJARiKnVrUz42f1P7A4wJoTO</recordid><startdate>20240101</startdate><enddate>20240101</enddate><creator>Dehghan, Bahar</creator><creator>Rostampour, Noushin</creator><creator>Sedighi, Mohsen</creator><creator>Saryazdi, Mohammad Hosseini</creator><creator>Rizi, Mohammad Jokar</creator><creator>Mostofizadeh, Neda</creator><creator>Hashemipour, Mahin</creator><creator>Khoshhali, Mehri</creator><general>Springer Netherlands</general><general>Springer Nature B.V</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FD</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M7Z</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0003-3684-8196</orcidid></search><sort><creationdate>20240101</creationdate><title>Evaluation of cardiac findings in mucopolysaccharidosis</title><author>Dehghan, Bahar ; Rostampour, Noushin ; Sedighi, Mohsen ; Saryazdi, Mohammad Hosseini ; Rizi, Mohammad Jokar ; Mostofizadeh, Neda ; Hashemipour, Mahin ; Khoshhali, Mehri</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c326t-d08ca90abedff8efa01dc13ed1adf39e492514cbb9cfddf6771aee265a4cdddd3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Age</topic><topic>Blood pressure</topic><topic>Body mass index</topic><topic>Cardiac Imaging</topic><topic>Cardiology</topic><topic>Complications</topic><topic>Congenital diseases</topic><topic>Cross-sectional studies</topic><topic>Echocardiography</topic><topic>Enzymes</topic><topic>Genetic disorders</topic><topic>Heart</topic><topic>Heart diseases</topic><topic>Imaging</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Metabolic disorders</topic><topic>Mucopolysaccharidosis</topic><topic>Original Paper</topic><topic>Pulmonary arteries</topic><topic>Radiology</topic><topic>Regurgitation</topic><topic>Research centers</topic><topic>Stenosis</topic><topic>Variance analysis</topic><topic>Ventricle</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Dehghan, Bahar</creatorcontrib><creatorcontrib>Rostampour, Noushin</creatorcontrib><creatorcontrib>Sedighi, Mohsen</creatorcontrib><creatorcontrib>Saryazdi, Mohammad Hosseini</creatorcontrib><creatorcontrib>Rizi, Mohammad Jokar</creatorcontrib><creatorcontrib>Mostofizadeh, Neda</creatorcontrib><creatorcontrib>Hashemipour, Mahin</creatorcontrib><creatorcontrib>Khoshhali, Mehri</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Technology Research Database</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>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Biochemistry Abstracts 1</collection><collection>Biotechnology and BioEngineering Abstracts</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><jtitle>The international journal of cardiovascular imaging</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Dehghan, Bahar</au><au>Rostampour, Noushin</au><au>Sedighi, Mohsen</au><au>Saryazdi, Mohammad Hosseini</au><au>Rizi, Mohammad Jokar</au><au>Mostofizadeh, Neda</au><au>Hashemipour, Mahin</au><au>Khoshhali, Mehri</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Evaluation of cardiac findings in mucopolysaccharidosis</atitle><jtitle>The international journal of cardiovascular imaging</jtitle><stitle>Int J Cardiovasc Imaging</stitle><addtitle>Int J Cardiovasc Imaging</addtitle><date>2024-01-01</date><risdate>2024</risdate><volume>40</volume><issue>1</issue><spage>73</spage><epage>78</epage><pages>73-78</pages><issn>1875-8312</issn><issn>1569-5794</issn><eissn>1875-8312</eissn><eissn>1573-0743</eissn><abstract>Purpose
Mucopolysaccharidoses (MPS) are a group of rare genetic diseases and heart involvement is one of the important conflicts in most types, which may cause serious complications. We used M-Mode and two-dimensional speckle tracking echocardiography (2D-STE) to explore cardiovascular involvements in MPS patients.
Method
The present cross-sectional study investigated the frequency of cardiac involvements in MPS patients. Included participants were MPS types I, II, III, IV, and VI who underwent specialized echocardiography exams to assess valvular function, systolic and diastolic function, left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS).
Results
35 patients were enrolled in this study. The total mean age of patients was 9.58 ± 5.11 years and 71.4% were male. Type IV (40%) and type III (31.4%) were the most frequent MPS. Although LVEF did not differ notably among MPS types, GLS was significantly different (p = 0.029). Mitral regurgitation was observed remarkably more in MPS type III (p = 0.001) while mitral stenosis was more common in type III (p = 0.007). There was a significant association between LVEF and GLS (β= -0.662; p = 0.025) and between LVEF and MPS type (β = 1.82; p = 0.025) when adjusted for GLS.
Conclusion
Cardiac complications are very common and are one of the most important causes of death in MPS patients. 2D-STE seems to be superior to M-Mode for detection of early and subclinical cardiac dysfunction in MPS patients.</abstract><cop>Dordrecht</cop><pub>Springer Netherlands</pub><pmid>37845409</pmid><doi>10.1007/s10554-023-02983-y</doi><tpages>6</tpages><orcidid>https://orcid.org/0000-0003-3684-8196</orcidid></addata></record> |
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| subjects | Age Blood pressure Body mass index Cardiac Imaging Cardiology Complications Congenital diseases Cross-sectional studies Echocardiography Enzymes Genetic disorders Heart Heart diseases Imaging Medicine Medicine & Public Health Metabolic disorders Mucopolysaccharidosis Original Paper Pulmonary arteries Radiology Regurgitation Research centers Stenosis Variance analysis Ventricle |
| title | Evaluation of cardiac findings in mucopolysaccharidosis |
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