Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan Syndrome in Children
Cardiomyopathy is a complication in adults with Marfan syndrome (MFS). Early recognition of MFS patients at high risk of cardiomyopathy could impact monitoring and treatment. Abnormal ventricular strain has been associated with impaired ventricular function among adults with MFS but remains understu...
Gespeichert in:
| Veröffentlicht in: | Pediatric cardiology 2023-10, Vol.44 (7), p.1536-1545 |
|---|---|
| Hauptverfasser: | , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 1545 |
|---|---|
| container_issue | 7 |
| container_start_page | 1536 |
| container_title | Pediatric cardiology |
| container_volume | 44 |
| creator | Connell, Patrick S. Morris, Shaine A. Doan, Tam T. Weigand, Justin |
| description | Cardiomyopathy is a complication in adults with Marfan syndrome (MFS). Early recognition of MFS patients at high risk of cardiomyopathy could impact monitoring and treatment. Abnormal ventricular strain has been associated with impaired ventricular function among adults with MFS but remains understudied in children. We retrospectively analyzed a cohort of patients with MFS undergoing cardiac magnetic resonance imaging (CMR) performed in 2003–2018 at age |
| doi_str_mv | 10.1007/s00246-023-03232-8 |
| format | Article |
| fullrecord | <record><control><sourceid>gale_proqu</sourceid><recordid>TN_cdi_proquest_miscellaneous_2852635416</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><galeid>A761355546</galeid><sourcerecordid>A761355546</sourcerecordid><originalsourceid>FETCH-LOGICAL-c414t-803cbb3cbee2d5e283d0bb10ec916cfeebfb4e91f644ede1ee5e1e18b742ea493</originalsourceid><addsrcrecordid>eNp9kc2KFDEUhYMoTjv6Ai4k4MZNjfmrn142PToKLQqjbkOSuunOkErGJIX0A_jek7ZGQRQJyQ0337mEcxB6TskFJaR_nQlhomsI4w3hjLNmeIBWVNQLXff0IVoR2rOGdIKfoSc53xBCBjK0j9EZ71vSsq5foR8bHWKalMc7sAV_hVCSM7NXCV-XpFzA25gSeFUg4--uHP7mLo_ZzsEUFwPWc8EhFryJqTiDP6lyiD7uj7gO-qCSVQFfH8OY4gSn1vbg_JggPEWPrPIZnt3Xc_Tl7ZvP23fN7uPV--1m1xhBRWkGwo3WdQOwsQU28JFoTQmYNe2MBdBWC1hT2wkBI1CAth500L1goMSan6NXy9zbFL_NkIucXDbgvQoQ5yzZUF3hraBdRV8u6F55kC7YWO0wJ1xu-o7ytm3Fibr4B1XXCJMzMYB1tf-HgC0Ck2LOCay8TW5S6SgpkadU5ZKqrKnKn6nKoYpe3H971hOMvyW_YqwAX4Bcn8IekryJcwrVyv-NvQN6Uq74</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2852635416</pqid></control><display><type>article</type><title>Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan Syndrome in Children</title><source>Springer Nature - Complete Springer Journals</source><creator>Connell, Patrick S. ; Morris, Shaine A. ; Doan, Tam T. ; Weigand, Justin</creator><creatorcontrib>Connell, Patrick S. ; Morris, Shaine A. ; Doan, Tam T. ; Weigand, Justin</creatorcontrib><description>Cardiomyopathy is a complication in adults with Marfan syndrome (MFS). Early recognition of MFS patients at high risk of cardiomyopathy could impact monitoring and treatment. Abnormal ventricular strain has been associated with impaired ventricular function among adults with MFS but remains understudied in children. We retrospectively analyzed a cohort of patients with MFS undergoing cardiac magnetic resonance imaging (CMR) performed in 2003–2018 at age < 19 years. Correlations were evaluated between initial global circumferential strain (GCS) and global longitudinal strain (GLS) and the outcomes of left ventricular ejection fraction (LVEF), aortic root
z
-score, and vertebral artery tortuosity index corrected for height (VTI-h), all measured from CMR, using Spearman correlation. In those with serial CMR, the ability of ventricular strain to predict development of abnormal LVEF within a 5-year period was assessed. A total of 31 subjects were included (median age at initial CMR 13.5 years, Q1Q3 10.7–16.2 years), with 48% (
n
= 15) having LVEF < 55%. Worse GCS and worse GLS were associated with lower LVEF (
ρ
= − 0.629,
p
< 0.001 and
ρ
= − 0.411,
p
= 0.030, respectively). A clinical cutoff of GCS = − 34% predicted LVEF < 55% with sensitivity = 80% and specificity = 50%. Neither GCS nor GLS was associated with aortic root
z
-score (GCS:
p
= 0.524; GLS:
p
= 0.624) nor VTI-h (GCS:
p
= 0.949; GLS:
p
= 0.593). Of those with LVEF ≥ 55%, initial GCS and GLS did not differ between those with later normal versus abnormal LVEF (GCS:
p
= 0.505; GLS:
p
= 0.232). In this cohort, abnormal LV strain was associated with abnormal LVEF, but not with aortic dilation or low LVEF within the 5 years post-CMR.</description><identifier>ISSN: 0172-0643</identifier><identifier>EISSN: 1432-1971</identifier><identifier>DOI: 10.1007/s00246-023-03232-8</identifier><identifier>PMID: 37505267</identifier><language>eng</language><publisher>New York: Springer US</publisher><subject>Cardiac patients ; Cardiac Surgery ; Cardiology ; Care and treatment ; Marfan syndrome ; Medicine ; Medicine & Public Health ; Vascular Surgery</subject><ispartof>Pediatric cardiology, 2023-10, Vol.44 (7), p.1536-1545</ispartof><rights>The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 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 Science+Business Media, LLC, part of Springer Nature.</rights><rights>COPYRIGHT 2023 Springer</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c414t-803cbb3cbee2d5e283d0bb10ec916cfeebfb4e91f644ede1ee5e1e18b742ea493</citedby><cites>FETCH-LOGICAL-c414t-803cbb3cbee2d5e283d0bb10ec916cfeebfb4e91f644ede1ee5e1e18b742ea493</cites><orcidid>0000-0002-0016-8140 ; 0000-0002-8256-1535 ; 0000-0003-4720-7730 ; 0000-0002-8056-0934</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/s00246-023-03232-8$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00246-023-03232-8$$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/37505267$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Connell, Patrick S.</creatorcontrib><creatorcontrib>Morris, Shaine A.</creatorcontrib><creatorcontrib>Doan, Tam T.</creatorcontrib><creatorcontrib>Weigand, Justin</creatorcontrib><title>Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan Syndrome in Children</title><title>Pediatric cardiology</title><addtitle>Pediatr Cardiol</addtitle><addtitle>Pediatr Cardiol</addtitle><description>Cardiomyopathy is a complication in adults with Marfan syndrome (MFS). Early recognition of MFS patients at high risk of cardiomyopathy could impact monitoring and treatment. Abnormal ventricular strain has been associated with impaired ventricular function among adults with MFS but remains understudied in children. We retrospectively analyzed a cohort of patients with MFS undergoing cardiac magnetic resonance imaging (CMR) performed in 2003–2018 at age < 19 years. Correlations were evaluated between initial global circumferential strain (GCS) and global longitudinal strain (GLS) and the outcomes of left ventricular ejection fraction (LVEF), aortic root
z
-score, and vertebral artery tortuosity index corrected for height (VTI-h), all measured from CMR, using Spearman correlation. In those with serial CMR, the ability of ventricular strain to predict development of abnormal LVEF within a 5-year period was assessed. A total of 31 subjects were included (median age at initial CMR 13.5 years, Q1Q3 10.7–16.2 years), with 48% (
n
= 15) having LVEF < 55%. Worse GCS and worse GLS were associated with lower LVEF (
ρ
= − 0.629,
p
< 0.001 and
ρ
= − 0.411,
p
= 0.030, respectively). A clinical cutoff of GCS = − 34% predicted LVEF < 55% with sensitivity = 80% and specificity = 50%. Neither GCS nor GLS was associated with aortic root
z
-score (GCS:
p
= 0.524; GLS:
p
= 0.624) nor VTI-h (GCS:
p
= 0.949; GLS:
p
= 0.593). Of those with LVEF ≥ 55%, initial GCS and GLS did not differ between those with later normal versus abnormal LVEF (GCS:
p
= 0.505; GLS:
p
= 0.232). In this cohort, abnormal LV strain was associated with abnormal LVEF, but not with aortic dilation or low LVEF within the 5 years post-CMR.</description><subject>Cardiac patients</subject><subject>Cardiac Surgery</subject><subject>Cardiology</subject><subject>Care and treatment</subject><subject>Marfan syndrome</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Vascular Surgery</subject><issn>0172-0643</issn><issn>1432-1971</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><recordid>eNp9kc2KFDEUhYMoTjv6Ai4k4MZNjfmrn142PToKLQqjbkOSuunOkErGJIX0A_jek7ZGQRQJyQ0337mEcxB6TskFJaR_nQlhomsI4w3hjLNmeIBWVNQLXff0IVoR2rOGdIKfoSc53xBCBjK0j9EZ71vSsq5foR8bHWKalMc7sAV_hVCSM7NXCV-XpFzA25gSeFUg4--uHP7mLo_ZzsEUFwPWc8EhFryJqTiDP6lyiD7uj7gO-qCSVQFfH8OY4gSn1vbg_JggPEWPrPIZnt3Xc_Tl7ZvP23fN7uPV--1m1xhBRWkGwo3WdQOwsQU28JFoTQmYNe2MBdBWC1hT2wkBI1CAth500L1goMSan6NXy9zbFL_NkIucXDbgvQoQ5yzZUF3hraBdRV8u6F55kC7YWO0wJ1xu-o7ytm3Fibr4B1XXCJMzMYB1tf-HgC0Ck2LOCay8TW5S6SgpkadU5ZKqrKnKn6nKoYpe3H971hOMvyW_YqwAX4Bcn8IekryJcwrVyv-NvQN6Uq74</recordid><startdate>20231001</startdate><enddate>20231001</enddate><creator>Connell, Patrick S.</creator><creator>Morris, Shaine A.</creator><creator>Doan, Tam T.</creator><creator>Weigand, Justin</creator><general>Springer US</general><general>Springer</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-0016-8140</orcidid><orcidid>https://orcid.org/0000-0002-8256-1535</orcidid><orcidid>https://orcid.org/0000-0003-4720-7730</orcidid><orcidid>https://orcid.org/0000-0002-8056-0934</orcidid></search><sort><creationdate>20231001</creationdate><title>Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan Syndrome in Children</title><author>Connell, Patrick S. ; Morris, Shaine A. ; Doan, Tam T. ; Weigand, Justin</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c414t-803cbb3cbee2d5e283d0bb10ec916cfeebfb4e91f644ede1ee5e1e18b742ea493</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Cardiac patients</topic><topic>Cardiac Surgery</topic><topic>Cardiology</topic><topic>Care and treatment</topic><topic>Marfan syndrome</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Vascular Surgery</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Connell, Patrick S.</creatorcontrib><creatorcontrib>Morris, Shaine A.</creatorcontrib><creatorcontrib>Doan, Tam T.</creatorcontrib><creatorcontrib>Weigand, Justin</creatorcontrib><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>Connell, Patrick S.</au><au>Morris, Shaine A.</au><au>Doan, Tam T.</au><au>Weigand, Justin</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan Syndrome in Children</atitle><jtitle>Pediatric cardiology</jtitle><stitle>Pediatr Cardiol</stitle><addtitle>Pediatr Cardiol</addtitle><date>2023-10-01</date><risdate>2023</risdate><volume>44</volume><issue>7</issue><spage>1536</spage><epage>1545</epage><pages>1536-1545</pages><issn>0172-0643</issn><eissn>1432-1971</eissn><abstract>Cardiomyopathy is a complication in adults with Marfan syndrome (MFS). Early recognition of MFS patients at high risk of cardiomyopathy could impact monitoring and treatment. Abnormal ventricular strain has been associated with impaired ventricular function among adults with MFS but remains understudied in children. We retrospectively analyzed a cohort of patients with MFS undergoing cardiac magnetic resonance imaging (CMR) performed in 2003–2018 at age < 19 years. Correlations were evaluated between initial global circumferential strain (GCS) and global longitudinal strain (GLS) and the outcomes of left ventricular ejection fraction (LVEF), aortic root
z
-score, and vertebral artery tortuosity index corrected for height (VTI-h), all measured from CMR, using Spearman correlation. In those with serial CMR, the ability of ventricular strain to predict development of abnormal LVEF within a 5-year period was assessed. A total of 31 subjects were included (median age at initial CMR 13.5 years, Q1Q3 10.7–16.2 years), with 48% (
n
= 15) having LVEF < 55%. Worse GCS and worse GLS were associated with lower LVEF (
ρ
= − 0.629,
p
< 0.001 and
ρ
= − 0.411,
p
= 0.030, respectively). A clinical cutoff of GCS = − 34% predicted LVEF < 55% with sensitivity = 80% and specificity = 50%. Neither GCS nor GLS was associated with aortic root
z
-score (GCS:
p
= 0.524; GLS:
p
= 0.624) nor VTI-h (GCS:
p
= 0.949; GLS:
p
= 0.593). Of those with LVEF ≥ 55%, initial GCS and GLS did not differ between those with later normal versus abnormal LVEF (GCS:
p
= 0.505; GLS:
p
= 0.232). In this cohort, abnormal LV strain was associated with abnormal LVEF, but not with aortic dilation or low LVEF within the 5 years post-CMR.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>37505267</pmid><doi>10.1007/s00246-023-03232-8</doi><tpages>10</tpages><orcidid>https://orcid.org/0000-0002-0016-8140</orcidid><orcidid>https://orcid.org/0000-0002-8256-1535</orcidid><orcidid>https://orcid.org/0000-0003-4720-7730</orcidid><orcidid>https://orcid.org/0000-0002-8056-0934</orcidid></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0172-0643 |
| ispartof | Pediatric cardiology, 2023-10, Vol.44 (7), p.1536-1545 |
| issn | 0172-0643 1432-1971 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_2852635416 |
| source | Springer Nature - Complete Springer Journals |
| subjects | Cardiac patients Cardiac Surgery Cardiology Care and treatment Marfan syndrome Medicine Medicine & Public Health Vascular Surgery |
| title | Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan Syndrome in Children |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-30T19%3A14%3A57IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_proqu&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Abnormal%20Left%20Ventricular%20Strain%20Correlates%20with%20Left%20Ventricular%20Dysfunction%20but%20not%20Aortic%20Pathology%20in%20Marfan%20Syndrome%20in%20Children&rft.jtitle=Pediatric%20cardiology&rft.au=Connell,%20Patrick%20S.&rft.date=2023-10-01&rft.volume=44&rft.issue=7&rft.spage=1536&rft.epage=1545&rft.pages=1536-1545&rft.issn=0172-0643&rft.eissn=1432-1971&rft_id=info:doi/10.1007/s00246-023-03232-8&rft_dat=%3Cgale_proqu%3EA761355546%3C/gale_proqu%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2852635416&rft_id=info:pmid/37505267&rft_galeid=A761355546&rfr_iscdi=true |