{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T16:59:11Z","timestamp":1793120351425,"version":"4.5.0"},"reference-count":38,"publisher":"Springer Science and Business Media LLC","issue":"6","license":[{"start":{"date-parts":[[2020,12,23]],"date-time":"2020-12-23T00:00:00Z","timestamp":1608681600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,12,23]],"date-time":"2020-12-23T00:00:00Z","timestamp":1608681600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Projekt DEAL"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Eur Radiol"],"published-print":{"date-parts":[[2021,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Objectives<\/jats:title>\n                <jats:p>To evaluate systolic cardiac dysfunction in paediatric MFS patients with chest wall deformity using cardiac magnetic resonance (CMR) imaging and feature-tracking strain analysis.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Forty paediatric MFS patients (16 \u00b1 3 years, range 8\u221222 years) and 20 age-matched healthy controls (16 \u00b1 4 years, range 11\u221224 years) were evaluated retrospectively. Biventricular function and volumes were determined using cine sequences. Feature-tracking CMR was used to assess global systolic longitudinal (GLS), circumferential (GCS) and radial strain (GRS). A dedicated balanced turbo field echo sequence was used to quantify chest wall deformity by measuring the Haller index (HI).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>LV volumes and ejection fraction (EF) were similar in MFS patients and controls. There was a trend for lower right ventricular (RV) volume (75 \u00b1 17 vs. 81 \u00b1 10 ml\/m<jats:sup>2<\/jats:sup>, <jats:italic>p<\/jats:italic> = 0.08), RV stroke volume (41 \u00b1 12 vs. 50 \u00b1 5 ml\/m<jats:sup>2<\/jats:sup>, <jats:italic>p<\/jats:italic> &lt; 0.001) and RVEF (55 \u00b1 10 vs. 62 \u00b1 6%, <jats:italic>p<\/jats:italic> &lt; 0.01) in MFS patients. A subgroup of MFS patients had an increased HI compared to controls (4.6 \u00b1 1.7 vs. 2.6 \u00b1 0.3, <jats:italic>p<\/jats:italic> &lt; 0.001). They demonstrated a reduced RVEF compared to MFS patients without chest wall deformity (50 \u00b1 11% vs. 58 \u00b1 8%, <jats:italic>p<\/jats:italic> = 0.01) and controls (<jats:italic>p<\/jats:italic> &lt; 0.001). LV GLS was attenuated when HI \u2265 3.25 (- 16 \u00b1 2 vs. - 18 \u00b1 3%, <jats:italic>p =<\/jats:italic> 0.03), but not GCS and GRS. LV GLS (<jats:italic>p &lt;<\/jats:italic> 0.01) and GCS (<jats:italic>p<\/jats:italic> &lt; 0.0001) were attenuated in MFS patients compared to controls, but not GRS (<jats:italic>p<\/jats:italic> = 0.31). RV GLS was attenuated in MFS patients compared to controls (- 21 \u00b1 3 vs. - 23 \u00b1 3%, <jats:italic>p<\/jats:italic> &lt; 0.05).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>Chest wall deformity in paediatric MFS patients is associated with reduced RV volume, ejection fraction and GLS. Feature-tracking CMR also indicates impairment of systolic LV function in paediatric MFS patients.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Key Points<\/jats:title>\n                <jats:p><jats:italic>\u2022 Paediatric Marfan patients demonstrate reduced RV volume and ejection fraction compared to healthy controls.<\/jats:italic><\/jats:p>\n                <jats:p><jats:italic>\u2022 A concordant attenuation in RV global longitudinal strain was observed in Marfan patients, while the RV global circumferential strain was increased, indicating a possible compensatory mechanism.<\/jats:italic><\/jats:p>\n                <jats:p><jats:italic>\u2022 Subgroup analyses demonstrated alterations in RV ejection fraction and RV\/LV global strain parameters, indicating a possible association of severe chest wall deformity with biventricular dysfunction in paediatric Marfan patients.<\/jats:italic><\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00330-020-07616-9","type":"journal-article","created":{"date-parts":[[2020,12,23]],"date-time":"2020-12-23T08:02:33Z","timestamp":1608710553000},"page":"3973-3982","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Impact of chest wall deformity on cardiac function by CMR and feature-tracking strain analysis in paediatric patients with Marfan syndrome"],"prefix":"10.1007","volume":"31","author":[{"given":"Hy Van","family":"Lam","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Groth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Mir","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Bannas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gunnar K.","family":"Lund","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte M.","family":"Jahnke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malte","family":"Warncke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai-Jonathan","family":"Maas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerhard","family":"Adam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen","family":"Herrmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6999-2787","authenticated-orcid":false,"given":"Enver","family":"Tahir","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,12,23]]},"reference":[{"key":"7616_CR1","doi-asserted-by":"publisher","first-page":"481","DOI":"10.1146\/annurev.med.51.1.481","volume":"51","author":"RE Pyeritz","year":"2000","unstructured":"Pyeritz RE (2000) The Marfan syndrome. 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