{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T13:33:47Z","timestamp":1777901627413,"version":"3.51.4"},"reference-count":38,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,12,18]],"date-time":"2022-12-18T00:00:00Z","timestamp":1671321600000},"content-version":"vor","delay-in-days":78,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82070243"],"award-info":[{"award-number":["82070243"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2022,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>To assess the long\u2010term prognostic value of cardiac catheterization and acute vasodilator testing (AVT) with inhaled iloprost in children with idiopathic pulmonary arterial hypertension (IPAH).\u00a0Data on 81 consecutive children with IPAH referred to our center who underwent cardiac catheterization and AVT between June 2008 and August 2019 were collected. The correlation between the invasive hemodynamic data and transplant\u2010free survival was analyzed. Twenty\u2010four patients died and 1 underwent lung transplantation during a median follow\u2010up of 3.8 years, with a 5\u2010year transplant\u2010free survival rate of 64.9%. Univariate analysis showed that predictors associated with improved survival included a lower pulmonary vascular resistance index (PVRI), PVRI\/systemic vascular resistance index (SVRI), mean pulmonary arterial pressure (mPAP)\/mean systemic arterial pressure, mean right atrial pressure, and a higher cardiac index (CI), mixed systemic venous oxygen saturations (SvO<jats:sub>2<\/jats:sub>), and acute vasodilator response (AVR) according to the Barst criteria (decrease in mPAP and PVRI\/SVRI ratio of &gt;20% without a decrease in CI). In multivariate Cox regression analysis, Barst AVR and SvO<jats:sub>2<\/jats:sub> were independently related to transplant\u2010free survival. Multiple hemodynamic variables from cardiac catheterization and AVT with inhaled iloprost have important prognostic value for long\u2010term survival in children with IPAH, of which pulmonary vasoreactivity defined by the Barst criteria and SvO<jats:sub>2<\/jats:sub> are independent prognostic factors.<\/jats:p>","DOI":"10.1002\/pul2.12169","type":"journal-article","created":{"date-parts":[[2022,12,9]],"date-time":"2022-12-09T11:23:40Z","timestamp":1670585020000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Long\u2010term prognostic value of cardiac catheterization and acute vasodilator testing with inhaled iloprost in pediatric idiopathic pulmonary arterial hypertension"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8573-8236","authenticated-orcid":false,"given":"Chen","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Pediatric Cardiology, Beijing Anzhen Hospital Capital Medical University  Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Konstantinos","family":"Dimopoulos","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2649-6775","authenticated-orcid":false,"given":"Qiangqiang","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Beijing Anzhen Hospital Capital Medical University  Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hong","family":"Gu","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Beijing Anzhen Hospital Capital Medical University  Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,12,18]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(03)13167-4"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-115-5-343"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.99.9.1197"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2019.06.022"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehac237"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.104.488486"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehr282"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2016.01.015"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/ped.13161"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1086\/685102"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00169608"},{"key":"e_1_2_10_13_1","doi-asserted-by":"crossref","first-page":"i23","DOI":"10.1136\/heartjnl-2014-307340","article-title":"Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary vascular disease. 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