{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T07:10:52Z","timestamp":1780384252545,"version":"3.54.1"},"reference-count":39,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"11","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">Marfan syndrome is characterized by aortic root dilation, beginning in childhood. Data about aortic pulsatile hemodynamics and stiffness in pediatric age are currently lacking.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              In 51 young patients with Marfan syndrome (12.0\u00b13.3\u00a0years), carotid tonometry was performed for the measurement of central pulse pressure, pulse pressure amplification, and aortic stiffness (carotid\u2010femoral pulse wave velocity). Patients underwent an echocardiogram at baseline and at 1\u00a0year follow\u2010up and a genetic evaluation. Pathogenetic fibrillin\u20101 mutations were classified between \u201cdominant negative\u201d and \u201chaploinsufficient.\u201d The hemodynamic parameters of patients were compared with those of 80 sex, age, blood pressure, and heart\u2010rate matched controls. Central pulse pressure was significantly higher (38.3\u00b112.3 versus 33.6\u00b17.8\u00a0mm\u00a0Hg;\n              <jats:italic>P<\/jats:italic>\n              =0.009), and pulse pressure amplification was significantly reduced in Marfan than controls (17.9\u00b115.3% versus 32.3\u00b117.4%;\n              <jats:italic>P<\/jats:italic>\n              &lt;0.0001). Pulse wave velocity was not significantly different between Marfan and controls (4.98\u00b11.00 versus 4.75\u00b10.67\u00a0m\/s). In the Marfan group, central pulse pressure and pulse pressure amplification were independently associated with aortic diameter at the sinuses of Valsalva (respectively, \u03b2=0.371,\n              <jats:italic>P<\/jats:italic>\n              =0.010; \u03b2=\u22120.271,\n              <jats:italic>P<\/jats:italic>\n              =0.026). No significant difference in hemodynamic parameters was found according to fibrillin\u20101 genotype. Patients who increased aortic Z\u2010scores at 1\u2010year follow\u2010up presented a higher central pulse pressure than the remaining (42.7\u00b114.2 versus 32.3\u00b15.9\u00a0mm\u00a0Hg;\n              <jats:italic>P<\/jats:italic>\n              =0.004).\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">Central pulse pressure and pulse pressure amplification were impaired in pediatric Marfan syndrome, and associated with aortic root diameters, whereas aortic pulse wave velocity was similar to that of a general pediatric population. An increased central pulse pressure was present among patients whose aortic dilatation worsened at 1\u2010year follow\u2010up.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.117.006815","type":"journal-article","created":{"date-parts":[[2017,11,8]],"date-time":"2017-11-08T01:10:41Z","timestamp":1510103441000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":13,"title":["Impaired Central Pulsatile Hemodynamics in Children and Adolescents With Marfan Syndrome"],"prefix":"10.1161","volume":"6","author":[{"given":"Andrea","family":"Grillo","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Neural and Metabolic Sciences, Istituto Auxologico Italiano, Milan, Italy"},{"name":"Department of Medicine and Surgery, University of Milano\u2010Bicocca, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Salvi","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Neural and Metabolic Sciences, Istituto Auxologico Italiano, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Marelli","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Rare Disease Center \u2018Marfan Clinic,\u2019 ASST Fatebenefratelli Sacco, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lan","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Peking University First Hospital, Beijing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Salvi","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Faini","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Neural and Metabolic Sciences, Istituto Auxologico Italiano, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuliana","family":"Trifir\u00f2","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, L. Salvini Hospital, Rho, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renzo","family":"Carretta","sequence":"additional","affiliation":[{"name":"Department of Medical, Surgical and Health Sciences, University of Trieste, Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Pini","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Rare Disease Center \u2018Marfan Clinic,\u2019 ASST Fatebenefratelli Sacco, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianfranco","family":"Parati","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Neural and Metabolic Sciences, Istituto Auxologico Italiano, Milan, Italy"},{"name":"Department of Medicine and Surgery, University of Milano\u2010Bicocca, Milan, 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