{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T06:49:09Z","timestamp":1776840549639,"version":"3.51.2"},"reference-count":32,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T00:00:00Z","timestamp":1776816000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Hypermobile Ehlers-Danlos syndrome (hEDS) is a connective tissue disorder that may involve the cardiovascular system. While mitral valve prolapse (MVP) and aortic root dilatation (ARD) have been documented in adults with hEDS, their prevalence in children remains unclear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This study evaluated 28 pediatric patients with hEDS and 28 age- and body surface area\u2013matched healthy controls using two-dimensional echocardiography, Doppler, Tissue Doppler Imaging (TDI), and Speckle Tracking Echocardiography (STE), including global longitudinal strain (GLS) analysis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Nine hEDS patients (32%) exhibited MVP. Although aortic root and ascending aorta Z-scores remained within the normal range, they were significantly higher in hEDS patients compared to controls. TDI revealed reduced late diastolic septal myocardial velocities (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.004). While left ventricular ejection fraction (EF) was preserved, hEDS patients showed significantly reduced GLS values (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&amp;lt;\u20090.001), indicating subclinical myocardial dysfunction. hEDS diagnosis correlated independently with reduced GLS (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.01), but not with EF.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>These findings suggest that cardiac alterations such as MVP can manifest in childhood and that subtle myocardial impairment can occur despite normal EF. STE and GLS analyses are valuable tools for the early detection of functional abnormalities. Routine cardiac follow-up in pediatric hEDS patients may help identify and monitor these early cardiovascular changes, potentially improving long-term outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fped.2026.1768520","type":"journal-article","created":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T05:50:53Z","timestamp":1776837053000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Morphological and functional echocardiographic findings in pediatric patients diagnosed with hypermobile Ehlers-Danlos syndrome"],"prefix":"10.3389","volume":"14","author":[{"given":"Walter","family":"Vignaroli","sequence":"first","affiliation":[{"name":"Department of Cardiac Surgery, San Carlo di Nancy Hospital, GVM Care & Research","place":["Rome, Italy"]},{"name":"Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gioia","family":"Mastromoro","sequence":"additional","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome","place":["Rome, Italy"]},{"name":"Department of Experimental Medicine, Sapienza University of Rome","place":["Rome, Italy"]},{"name":"Department of Laboratory Science, Research and Development Division, Ospedale Isola Tiberina-Gemelli Isola","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolina","family":"Putotto","sequence":"additional","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Filippo","family":"Camerota","sequence":"additional","affiliation":[{"name":"Physical Medicine and Rehabilitation, Sapienza University of Rome","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Celletti","sequence":"additional","affiliation":[{"name":"Department of Life Sciences, Health, and Health Profession, Link Campus University","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mauro","family":"Celli","sequence":"additional","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Celli","sequence":"additional","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Zambrano","sequence":"additional","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Carlomagno","sequence":"additional","affiliation":[{"name":"Department of Experimental Medicine, Sapienza University of Rome","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emanuele","family":"Monda","sequence":"additional","affiliation":[{"name":"Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania \u201cLuigi Vanvitelli\u201d, Monaldi Hospital","place":["Naples, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annapaola","family":"Cirillo","sequence":"additional","affiliation":[{"name":"Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania \u201cLuigi Vanvitelli\u201d, Monaldi Hospital","place":["Naples, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruno","family":"Marino","sequence":"additional","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paolo","family":"Versacci","sequence":"additional","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome","place":["Rome, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,4,22]]},"reference":[{"key":"B1","first-page":"351","article-title":"Connective tissue and its heritable disorders: molecular, genetic, and medical aspects.","volume-title":"The Ehlers-Danlos syndrome","author":"Royce","year":"1993"},{"key":"B2","doi-asserted-by":"publisher","first-page":"491","DOI":"10.1007\/s00441-010-1078-1","article-title":"In situ hybridization reveals that 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