{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T15:43:26Z","timestamp":1793029406220,"version":"4.5.0"},"reference-count":8,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T00:00:00Z","timestamp":1785456000000},"content-version":"vor","delay-in-days":30,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Neonates and infants with FOXF1 mutation develop alveolar capillary dysplasia with misalignment of pulmonary veins (ACD\/MPV), which is typically considered a uniformly fatal neonatal lung disorder due to marked hypoxemic respiratory failure with severe pulmonary hypertension (PH). Survival beyond infancy is exceedingly rare, with limited reports of FOXF1 genetic abnormalities in older children or adult cases of severe PH. Here, we report the case of a male patient with a pathogenic FOXF1 variant who presented with severe PH in early infancy whose disease stabilized and improved with early and aggressive PH therapy. The patient subsequently died at the age of 30 years, however, due to severe right ventricular failure due to the acute onset of pneumonia. This case highlights that survival into adulthood is feasible in patients with FOXF1\u2010related pulmonary vascular disease and supports the inclusion of FOXF1 in pulmonary hypertension genetic panels for subjects who develop PH across the lifespan.<\/jats:p>","DOI":"10.1002\/pul2.70378","type":"journal-article","created":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T10:10:47Z","timestamp":1785492647000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Survival Into Adulthood With FOXF1\u2010Associated Pulmonary Vascular Disease After Neonatal Onset"],"prefix":"10.1002","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0163-8658","authenticated-orcid":false,"given":"Khalifah A.","family":"Aldawsari","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Section of Cardiology University of Colorado Anschutz Medical School and Children's Hospital Colorado Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7292-2085","authenticated-orcid":false,"given":"Steven H.","family":"Abman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Pediatric Heart Lung Center University of Colorado Anschutz Medical School and Children's Hospital Colorado Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-6311-4828","authenticated-orcid":false,"given":"David","family":"Badesch","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences and Critical Care Medicine University of Colorado Anschutz Medical School Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2541-3402","authenticated-orcid":false,"given":"David Dunbar","family":"Ivy","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Section of Cardiology University of Colorado Anschutz Medical School and Children's Hospital Colorado Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,7,31]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9149(94)90420-0"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2009.05.005"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-024-49477-6"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01095-2024"},{"key":"e_1_2_9_6_1","doi-asserted-by":"crossref","unstructured":"J.Gomez\u2010Arroyo A. C.Houweling H. J.Bogaard et al. \u201cRole of Forkhead Box F1 in the Pathobiology of Pulmonary Arterial Hypertension \u201d Preprint bioRxiv September 22 2024 https:\/\/doi.org\/10.1101\/2024.09.18.611448.","DOI":"10.1101\/2024.09.18.611448"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41431-022-01159-x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23425"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-023-43039-y"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pul2.70378","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/pul2.70378","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pul2.70378","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T15:31:13Z","timestamp":1793028673000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pul2.70378"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":8,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,7]]}},"alternative-id":["10.1002\/pul2.70378"],"URL":"https:\/\/doi.org\/10.1002\/pul2.70378","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"value":"2045-8940","type":"print"},{"value":"2045-8940","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]},"assertion":[{"value":"2026-06-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-23","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-31","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70378"}}