{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,17]],"date-time":"2026-01-17T18:48:46Z","timestamp":1768675726521,"version":"3.49.0"},"reference-count":34,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2017,4,1]],"date-time":"2017-04-01T00:00:00Z","timestamp":1491004800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100006006","name":"ICIN Netherlands Heart Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006006","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Echocardiography"],"published-print":{"date-parts":[[2017,6]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Right ventricular (<jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content>) pressure overload in the context of pulmonary stenosis (<jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content>) has a much better prognosis than in the context of idiopathic pulmonary arterial hypertension (<jats:styled-content style=\"fixed-case\">iPAH<\/jats:styled-content>), which may be related to differences in global and regional <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> remodeling and systolic function. We compared <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> mechanics in children with <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> to those with <jats:styled-content style=\"fixed-case\">iPAH<\/jats:styled-content>, aiming to identify mechanisms and markers of <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> dysfunction.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Eighteen controls, 18 <jats:styled-content style=\"fixed-case\">iPAH<\/jats:styled-content> and 16 <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> patients were retrospectively studied. Age, <jats:styled-content style=\"fixed-case\">BSA<\/jats:styled-content>, and sex distribution were comparable. Two\u2010dimensional echocardiography, blood flow and tissue Doppler, and longitudinal <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> deformation were analyzed. <jats:styled-content style=\"fixed-case\">ANCOVA<\/jats:styled-content>\u2014including <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> systolic pressure (<jats:styled-content style=\"fixed-case\">RVSP<\/jats:styled-content>) and length as covariates\u2014was used to compare patient groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>RV systolic pressure was higher in <jats:styled-content style=\"fixed-case\">iPAH<\/jats:styled-content> vs <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> (96.8\u00b125.4 vs 75.4\u00b118.9\u00a0mm Hg, <jats:italic>P<\/jats:italic>=.011). Compared to controls, <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> patients showed mild dilation (<jats:italic>P<\/jats:italic>&lt;.01) and decreased longitudinal deformation (<jats:italic>P<\/jats:italic>&lt;.001) at the <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> apex. Compared to both <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> and controls, <jats:styled-content style=\"fixed-case\">iPAH<\/jats:styled-content> patients showed marked spherical <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> dilation (<jats:italic>P<\/jats:italic>&lt;.001), reduced global, <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> free wall and septal longitudinal deformation (<jats:styled-content style=\"fixed-case\">iPAH<\/jats:styled-content> \u221222.07%\u00b14.35% vs controls \u221228.18%\u00b11.69%; \u22129.98%\u00b14.30% vs \u221217.45%\u00b12.52%; <jats:italic>P<\/jats:italic>&lt;.001) and <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> postsystolic shortening (<jats:italic>P<\/jats:italic>&lt;.001). <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> transverse shortening (radial performance) was increased in <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> (31.75%\u00b110.35%; <jats:italic>P<\/jats:italic>&lt;.001) but reduced in <jats:styled-content style=\"fixed-case\">iPAH<\/jats:styled-content> (\u22121.62%\u00b111.11% vs controls 12.00%\u00b17.74%; <jats:italic>P<\/jats:italic>&lt;.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Children with <jats:styled-content style=\"fixed-case\">iPAH<\/jats:styled-content> demonstrate adverse global and regional <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> remodeling and mechanics compared to those with <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content>. Mechanisms of <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> systolic dysfunction in <jats:styled-content style=\"fixed-case\">iPAH<\/jats:styled-content> include decreased longitudinal deformation, decreased or absent transverse shortening, and postsystolic shortening. These markers may be useful to identify children at risk of <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> failure.<\/jats:p><\/jats:sec>","DOI":"10.1111\/echo.13529","type":"journal-article","created":{"date-parts":[[2017,4,4]],"date-time":"2017-04-04T00:55:14Z","timestamp":1491267314000},"page":"888-897","source":"Crossref","is-referenced-by-count":18,"title":["Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: Insights into mechanics of right ventricular dysfunction"],"prefix":"10.1111","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9713-378X","authenticated-orcid":false,"given":"Mieke M.P.","family":"Driessen","sequence":"first","affiliation":[{"name":"Department of Cardiology The Labatt Family Heart Center Hospital for Sick Children  Toronto ON Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto ON Canada"},{"name":"Interuniversity Cardiology Institute of the Netherlands\u2010Netherlands Heart Institute (ICIN\u2010NHI)  Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Folkert J.","family":"Meijboom","sequence":"additional","affiliation":[{"name":"Department of Cardiology University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wei","family":"Hui","sequence":"additional","affiliation":[{"name":"Department of Cardiology The Labatt Family Heart Center Hospital for Sick Children  Toronto ON Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreea","family":"Dragulescu","sequence":"additional","affiliation":[{"name":"Department of Cardiology The Labatt Family Heart Center Hospital for Sick Children  Toronto ON Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luc","family":"Mertens","sequence":"additional","affiliation":[{"name":"Department of Cardiology The Labatt Family Heart Center Hospital for Sick Children  Toronto ON Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark K.","family":"Friedberg","sequence":"additional","affiliation":[{"name":"Department of Cardiology The Labatt Family Heart Center Hospital for Sick Children  Toronto ON Canada"},{"name":"Department of Pediatrics University of Toronto  Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,4]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2013-305298"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2013-304958"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehl477"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.27.2.151"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2008.150086"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)61621-8"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2011.07.016"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1010-7940(98)00179-1"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2007.02.062"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.14814\/phy2.12833"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00056110"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2010.03.019"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1053\/euje.2000.0031"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.653576"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200604-547OC"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-001-0195-8"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/echo.13036"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2007.10.041"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2012.10.009"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehv680"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.72.3.515"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2007.02.005"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2014.09.002"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2010.216101"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2007.11.077"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1186\/1532-429X-12-35"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2005.04.028"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2005.09.054"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00129.2012"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9149(01)02159-2"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.883843"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.113.001071"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.113.000629"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1111\/echo.12912"}],"container-title":["Echocardiography"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fecho.13529","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/echo.13529","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T05:54:05Z","timestamp":1696312445000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/echo.13529"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,4]]},"references-count":34,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2017,6]]}},"alternative-id":["10.1111\/echo.13529"],"URL":"https:\/\/doi.org\/10.1111\/echo.13529","archive":["Portico"],"relation":{},"ISSN":["0742-2822","1540-8175"],"issn-type":[{"value":"0742-2822","type":"print"},{"value":"1540-8175","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,4]]}}}